Abstracts, 21st PANLAR Meeting

Quito, Ecuador, April 2019

JCR: Journal of Clinical Rheumatology: April 2019 - Volume 25 - Issue - p S1–S96
doi: 10.1097/RHU.0000000000001070
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Jarrat Jordan1, Kristen Sweet1, Matteo Cesaroni1, Loqmane Seridi1, Federico Zazzetti3, Peter Lipsky2, Shawn Rose1, Frédéric Baribaud1, Matthew Loza1, and Kim Campbell1. 1 Janssen Research & Development, LLC, Spring House, United States, 2 AMPEL BioSolutions, LLC, Charlottesville, United States, 3Janssen-Cilag, Buenos-Aires, Argentina.

Objectives: Treatment with ustekinumab (UST), an anti-IL-12/23, p40-neutralizing monoclonal antibody, improved global and organ-specific measures of disease activity in a randomized, placebo (PBO)-controlled trial of patients with active SLE (NCT02349061) 1. Type I interferon (IFN-I) and type II IFN (IFN-y) are elevated in a subset of SLE patients. Although targeting IFN-I (anifrolumab) has demonstrated inconsistent efficacy and a preliminary study with anti-IFN-y mAb (AMG811) failed to establish benefit2,3 we sought to determine if UST affects either pathway and if those effects correlated with a positive SRI-4 response at wk24.

Methods: A phase-2, PBO-controlled study enrolled 102 adults with seropositive SLE (SLICC criteria) and active disease (baseline SLEDAI score ≥6 and ≥1 BILAG A and/or ≥2 BILAG B scores) despite standard-of-care therapy1. Gene expression analysis using a 21 gene IFN-I gene signature (IGS)4 or IFN-y signature5 was performed by microarray analysis using whole blood PAXgene RNA samples. Serum IFN-y and IFN-α levels were assessed using MSD (IFN-y) and Quanterix (IFN-α).

Results: Serum IFN-y and IFN-α and the IGS were elevated at baseline in SLE compared to healthy controls (p<0.0001). IGS was increased in approximately 67% of the SLE patients at baseline. No decrease was observed with IFN-α protein or IGS levels after treatment with either UST or PBO. Whereas the proportion of patients achieving an SRI-4 response at wk24 was numerically greater in the IGS low patients (81.8% UST vs. 54.5% PBO) versus IGS high (48.6% UST vs. 20% PBO), the magnitude of the treatment effect (UST vs. PBO) was similar in both subsets (IGS low effect size= 27.3% vs. IGS high effect size = 28.6%). Despite similar baseline levels, UST-treated patients achieving an SRI-4 response at wk24 exhibited a significant decrease in IFN-y protein versus non-responders (p<0.05) at 4 and 8 wks and IFN-y gene signature at 4 wks (p<0.0001) and 24 wks (p<0.05) post-dosing.

Conclusions: In this SLE trial population which had significant upregulation of IFN-I at baseline, clinical response to UST was not associated with IFN-I reduction. In contrast, a significant decrease in IFN-y protein and gene signature was associated with UST response. These findings suggest that a broad population of SLE patients may respond to UST regardless of baseline IFN-I status. Moreover, UST may have affected TH1 responses in SLE since IFN-y levels decreased following treatment.


1VanVollenhoven RF. Lancet.2018:392:1330.

2Furie R. ArthritisRheumatol.2017;69:376.

3Boedigheimer M.J. LupusSciMed.2017;4: e000226.

4Yao Y. HumanGenomicsProteomics.2009; doi:10.4061/2009/374312.

5Welcher AA. Arthritis&Rheumatol.2015;67:2713.

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Martin Brom1, Ignacio Javier Gandino1, Marina Scolnik1, Valeria Scaglioni1, Maria Britos1, Carmen De Cunto1, and Enrique Roberto Soriano1. 1Hospital Italiano De Buenos Aires, Ciudad Autonoma De Buenos Aires, Argentina.

Objectives: To describe the clinical features, laboratory findings, treatment used and prognosis of Immunoglobulin A (IgA) Vasculitis (formerly Schönlein-Henoch purpura) in both pediatric and adult patients, and evaluate the differences between these groups, especially regarding renal outcome.

Methods: We performed a medical records review study on all patients with IgA vasculitis that were followed at a university hospital between 01/01/2000 and 05/01/2018.

We included all patients that fulfilled EULAR/PRINTO/PRES 2010 criteria, and collected and analyzed demographic, clinical, treatment and histopathologic information available on their electronic medical records.

We considered as pediatric patients those who were under 20 years when they were diagnosed.

Results: One-hundred eighteen patients were included, being 107 pediatric and 11 adults.

Clinical manifestations were similar in both groups except for kidney involvement that was more frequent in adults (36% vs 73%, p=0.02) while arthralgias (53% vs 18%, p=0.03) and scalp edema (42% vs 9%, p=0.03) were more frequent in children. Kidney biopsy was performed in 5/8 of the adult affected patients, with confirmatory histopathology for IgA vasculitis in all of them.

Since adults showed more severe clinical manifestations, significantly more corticosteroids and immunosuppressants were used in this group. As a consequence of the disease, 2 of the affected adults suffered from chronic kidney disease; one of them required kidney transplantation. No pediatric patient presented chronic kidney disease. Finally, 2 deaths occurred, both in the adult group, but only one was related to the disease.

Conclusions: Adult patients with IgA vasculitis suffered a more aggressive disease, with more renal involvement and required more intense treatment.

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Florencia Vivero1, Federico Campins1, Silvia Babini1, Pablo Malfante1, Diana Lancellotti1, Esteban Gándara1, Juan Ignacio Enghelmayer2, Victoria Basso1, Javier Sebastiani1, Adrián Gaser3, Silvia Quadrelli4, Patricia Aruj5, Carolina Isnardi6, Mónica Sacnun7, Norma Naval8, Viviana Moyano9, Soledad Altube10, Magdalena Romiti1, Mariana Lagrutta11, Brenda Varela12, Javier Abdala13, Paulin Francisco14, Gabriela Tabaj15, María Laura Alberti14, Santiago Auteri16, Joaquín Maritano17, Marcela Usandivaras18, Virginia Larivey19, Julio Fuertes Avila20, Germán Arce21, Ramiro Gomez2, Lilian Capone22, Luciana Molinari23, Matias Castro17, Victoria Avalos24, Alejandro Albiero25, Hernan Basilo Vigil26, Adriana Robles27, Franco Pacello28, Victoria Collado5, Guillermo Pons Estel29, Gabriela Manonelles30, Fabián Caro4, Maria Otaola6, and Marina Oliver1. 1 Hospital Privado De Comunidad, Mar Del Plata, Argentina, 2 Hospital de Clínicas, Buenos Aires, Argentina, 3 Instituto Diagnóstico Médico, Buenos Aires, Argentina, 4 Sanatorio Güemes, Buenos Aires, Argentina, 5 Instituto de investigaciones clínicas Dr. Lanari, Buenos Aires, Argentina, 6 Instituto de rehabilitación psicofísica, Buenos Aires, Argentina, 7 Hospital Provincial, Rosario, Argentina, 8 Hospital Ángel Padilla, San Miguel de Tucumán, Argentina, 9 Hospital Italiano, Córdoba, Argentina, 10 Hospital Municipal, Chivilcoy, Argentina, 11 Hospital Centenario, Rosario, Argentina, 12 Hospital Alemán, Buenos Aires, Argentina, 13 Hospital Central, Mendoza, Argentina, 14 Hospital Maria Ferrer, Buenos Aires, Argentina, 15 Hospital Cetrángolo, Buenos Aires, Argentina, 16 Sanatorio de la Mujer, Rosario, Argentina, 17 Hospital Italiano, Buenos Aires, Argentina, 18 Sanatorio 9 de julio, San Miguel de Tucumán, Argentina, 19 Hospital Sayago, Santa Fe, Argentina, 20 Clinica de artritis temprana, Cali, Colombia, 21 Grupo Gamma, Rosario, Argentina, 22 Instituto Vaccarezza, Buenos Aires, Argentina, 23 Clinica Roca, General Roca, Argentina, 24 Hospital Churruca, Buenos Aires, Argentina, 25 Sanatorio Allende, Córdoba, Argentina, 26 Hospital Tornú, Buenos Aires, Argentina, 27 Hospital San Bernardo, Salta, Argentina, 28 Unidad de Enf Autoinmunes, Paysandú, Uruguay, 29 Sanatorio Parque, Rosario, Argentina, 30Instituto Aike, Rio Gallegos, Argentina.

Objectives: The prevalence, clinical and radiological features along with the natural course of lung diseases (ILD) with autoimmune phenomena (Ai-ILD) as a whole are not entirely known as most of the studies available are limited to specific subsets of patients such as those with ILDs associated with well-defined connective tissue diseases (ILD-CTD), interstitial pneumonia with autoimmune features (IPAF) or ILD associated with anti-neutrophil cytoplasmatic antibodies (ILD-ANCA).

The aim of this study was to evaluate the clinical, radiological, functional, serological and therapeutic features of all patients diagnosed with Ai-ILD in a Latin America cohort.

Methods: We conducted a multicenter cohort study in hospitals from Argentina, Colombia, and Uruguay between January 2015 and April 2018. All the facilities selected to participate provided specialized care to patients with ILD by a multi-disciplinary team. Ai-ILD was defined as the presence of lung images suggestive of ILD in a chest high-resolution computed tomography (HRCT) associated with: 1) the presence of CTD defined according to international classification guidelines, or 2) the presence of IPAF according to the 2015 ERS/ATS criteria, or 3) the presence of ANCA by indirect immunofluorescence ELISA confirmed. All CT scans were evaluated by a specialist in radiology focused in chest disease, who was blinded to the participant’s medical history or suspected diagnosis. The following variables were collected: demographic features, co-morbidities, smoking, date of Ai-ILD diagnosis, serological data, ILD tomographic pattern, pulmonary function test (PFT), therapeutic schemes.

Results: During the study period 381 participants were enrolled. Female participants predominated (74%) with an average age of 58 years (SD 16). The most frequent diagnosis was ILD-CTD (85.3%) followed by IPAF (9.5%) and ILD-ANCA 3.5%. Regarding ILD-CTD, the most frequent condition was rheumatoid arthritis (RA) 31%, followed by systemic sclerosis (SSc) 28.6%. More frequent anti-bodies were anti-nuclear antibody (ANAs) (60,6%), rheumatoid factor (RF) (38%), anti-cyclic citrullinated peptide (CCP) (24%). Average baseline % forced vital capacity was 70% (SD 18.3). Forty-four percent of the participants presented severe restriction at the time of diagnosis, while 50.28% had mild restriction. The most frequent tomographic pattern was non-specific interstitial pneumonia (NSIP) accounting for 51.08% (95% CI 45.65 - 56.49) of all cases followed by usual interstitial pneumonia (UIP) with 21.36%. The most commonly used therapeutic regimen was steroids plus cyclophosphamide 30.1%, followed by azathioprine (20.3%). Corticosteroid pulses were used in (39.8%) of the participants.

Conclusions: Rheumatologists play an essential role in the selection of specific therapeutic strategies for the management of patients affected by Ai-ILD. EPIMAR is an important first step to better understand the characteristics of these patients. To the best of our knowledge, this is the first Latin American multicenter cohort study comprising facilities with experience in ILD management. Longitudinal studies are needed to better understand the outcome of patients treated with Ai-ILD

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Guillermo Valencia Pacheco1, Yumi E Nakazawa Ueji1, Darig Camara Cruz1, Gerardo J Perez Mendoza1, Angelica V Angulo Ramirez2, and Ricardo F López Villanueva3. 1 Laboratorio de Hematología. Centro de Investigaciones Regionales Dr. Hideyo Noguchi, de la Universidad Autonoma De Yucatan, Merida, Mexico, 2 Hospital Regional de Alta Especialidad de la Peninsula de Yucatan, Merida, México, 3Hospital General Regional (ISSSTE), Servicio de Salud de Yucatán, Merida, Mexico.

Objectives: Genetic factors such as copy number variation (CNV) of genes involved in the immune response, and epigenetic factors such as microRNA (miR), have been associated with the development of SLE. TLR7 is a innate immunity receptor, which has been implicated in the pathology of SLE.

Our objective was to correlate the expression of miR-3148 with the levels of mRNA and CNV of the TLR7 gene in Mayan women with SLE.

Methods: We studied 100 women diagnosed with SLE and 100 healthy women as a control group, all of Mayan origin. The expression of miR-3148, CNV, and mRNA of the TLR7 gene was determined by real-time PCR (Q-PCR) using TaqMan probes. The estimation of CNV, mRNA and miR was made by calculating 2-ΔΔCt.The association of CNV of the TLR7 gene with the disease was determined by the Odds Ratio (OR) with a confidence interval of 95%, and the results were contrasted by the χ square test. Correlation analysis was performed between CNV, mRNA, and miR-3148, using the Pearson test, considering as significant a value of p <0.05.

Results: We found that 17% of the patients presented more than two copies of the TLR7 gene, with respect to the controls. The analysis of association of CNV with the disease shows that the risk of developing SLE is almost 41 times higher in people with more than two copies with respect to those of two copies of the TLR7 gene (OR = 40.96, 95% CI 2.42 - 692.31, p=0.0001). Significant difference was observed in the CNV and mRNA expression of the TLR7 gene between patients and controls (p <0.0001). A correlation was found between CNV and TLR7 mRNA (r=0.4080, p=0.0016) and between miR-3148 and mRNA (r=0.5950, p=0.0002) in the patients; however, no correlation was observed between the CNV and expression of miR-3148.

Conclusions: These results indicate that Mayan women with more than two copies of TLR7 gene are more susceptible to developing SLE. The correlation between mRNA and CNV in patients supports the participation of the TLR7 gene in the disease. No correlation was observed between the CNV and miR-3148 in the patients indicating that TLR7 gene does not influence the expression of miR-3148.It is necessary to analyze other miR that regulate the expression of TLR7 gene. These data suggest that genetic and epigenetic variables may be risk markers to develop the disease in the Mayan population

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Carla Maldini1, Cintia Otaduy1, Florencia Beatriz Mollerach2, Marina Scolnik2, Belén Maria Virasoro3, Cecilia Pisoni3, Mercedes Croce4, María Hu4, Fabiola Natalia Camargo Serrudo5, Diana Dubinsky5, María de la Paz Leon6, Veronica Bellomio6, Daniela Flores Rengifo7, Sabrina Porta7, Fernanda Guzzanti8, Emma E Civit8, Ana Bertoli9, María José Lopez Perez9, Maximiliano Machado Escobar10, Veronica Savio11, Alejandra Babini11, Cecilia Alvarez12, Verónica Saurit12, Rosa Serrano Morales13, Cruz Lascano14, María Constanza Danielsen15, Mayra Etcheverry16, Adrian Estevez16, Marina Werner17, Laura Onetti17, Paula Alba1, and Carla Gobbi18. 1 Cátedra de Semiología UHMI 3 Hospital Córdoba FCM Universidad Nacional de Córdoba, Cordoba, Argentina, 2 Hospital Italiano de Buenos Aires, Ciudad Autónoma de Buenos Aires, Argentina, 3 Centro de Educación Médica e Investigaciones Clínicas Norberto Quirno (CEMIC), Ciudad Autónoma de Buenos Aires, Argentina, 4 Hospital Penna, Ciudad Autónoma de Buenos Aires, Argentina, 5 Sanatorio Guemes, Ciudad Autónoma de Buenos Aires, Argentina, 6 Hospital Padilla, San Miguel de Tucumán, Argentina, 7 Hospital J. M. Ramos Mejía, Ciudad Autónoma de Buenos Aires, Argentina, 8 Hospital El Carmen, Mendoza, Argentina, 9 Clínica Universitaria Reina Fabiola. Universidad Católica de Córdoba, Córdoba, Argentina, 10 Instituto de Maternidad y Ginecologia Nuestra Sra de las Mercedes, San Miguel de Tucumán, Argentina, 11 Hospital Italiano, Córdoba, Argentina, 12 Servicio de Reumatología, Hospital Privado Universitario de Córdoba, Córdoba, Argentina, 13 Centro Regional de Enfermedades Autoinmunes y Reumáticas (GO-CREAR)/Maternidad Oroño, Rosario, Argentina, 14 Hospital San Roque, San Salvador de Jujuy, Argentina, 15 Clínica del Pilar, Santiago del Estero, Argentina, 16 Hospital de Alta Complejidad El Cruce, Buenos Aires, Argentina, 17 Hospital Nacional de Clínicas, Córdoba, Argentina, 18Cátedra de Clínica Médica I, Hospital Córdoba FCM Universidad Nacional de Córdoba, Córdoba, Argentina.

Objectives: Neonatal lupus (NL) is a disease in children of mothers who have specific anti-Ro/La IgG autoantibodies by passive transplacental transfer. NL is characterized by skin and cardiac involvement, as well as cytopenias, hepatic or neurological manifestations. NL can be diagnosed intra-uterus or in the neonatal period, being self-limiting in several months or be irreversible. Objetives: to estimate the frequency of NL in children of mothers with anti-Ro/La in reference centers in the management of pregnancy and autoimmune diseases in Argentina, and to describe maternal and children features.

Methods: A descriptive multicenter study was conducted in reference centers in the management of autoimmune diseases and pregnancy in Argentina. Inclusion criteria were the presence of positive maternal serology anti-Ro/La and at least one pregnancy. Demographic and maternal-fetal clinical data were obtained from the clinical histories and each center completed a data collection form created for this study. We defined a NL case (born or not) who presented, pre and/or postpartum, characteristic skin lesions, cytopenias, cardiac involvement (CHB, endocardial fibroelastosis and dilated cardiomyopathy), hepatic or neurological manifestations. Ethnicity was classified using the GLADEL group definitions. NL frequency was calculated dividing the number NL cases by the number of mothers with positive anti-Ro/La serology.

Results: 18 reference centers in the management of autoimmune diseases and pregnancy participated in this study in 7 different geographic areas of Argentina (6 from Buenos Aires, 6 from Cordoba, 1 from Jujuy, 1 from Mendoza, 1 from Santa Fe, 1 from Santiago del Estero and 2 from Tucuman). 193 mothers with positive anti-Ro/La serology were included with 364 pregnancies. 19 NL cases were reported (10 diagnosed during pregnancy and 9 in the post-partum period.) The frequency of NL was estimated at 9.8% [95% CI 6.3-14.9] (CHB=6.2% [IC95% 3.5-10.7].) The most frequent manifestations were skin (n=7) and cardiac involvement (n=12). In 1 case, there was a history of NL in a previous pregnancy. Of the patients with CHB, 5 required a pacemaker.

Conclusions: In conclusion, the frequency of NL in our multicentric cohort is greater than in other international cohorts. Differences could be related to genetic/environmental factors as well as methodological limitations and selection bias.

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Carolina Isnardi1, Dafne Capelusnik1, Emilce Edith Schneeberger1, María de los Ángeles Correa1, Romina Lim2, María Hu2, María Janina Tapia3, Eduardo Kerzberg3, Eliana Soledad Blanco4, Federico Luján Benavidez4, Luciana Gonzalez Lucero5, Ana Lucía Barbaglia5, Marcela Bazzarelli6, Hernán Maldonado Ficco7, Silvana Perez8, Claudia Hartvig9, Mariana Salcedo10, and Gustavo Citera1. 1 Instituto De Rehabilitación Psicofísica, Caba, Argentina, 2 Hospital General de Agudos José M. Penna, CABA, Argentina, 3 Hospital General de Agudos José María Ramos Mejía, CABA, Argentina, 4 Hospital General de Agudos Dr. Cosme Argerich, CABA, Argentina, 5 Hospital Ángel C. Padilla, San Miguel de Tucumán, Argentina, 6 Hospital Interzonal General de Agudos Petrona V. de Cordero, Tigre, Argentina, 7 Hospital San Antonio de Padua, Río Cuarto, Argentina, 8 Hospital General de Agudos Dr. Enrique Tornú, CABA, Argentina, 9 Hospital Provincial de Rosario, Rosario, Argentina, 10San Nicolás, San Nicolás, Argentina.

Objectives: We have recently validated the Quality of Life-Rheumatoid Arthritis Scale (QOL-RA). Redundancy between questions N° 3 and N° 6 was observed, and besides QOL-RA was influenced by age and disease duration. For this reason, with the author’s permission, we have changed these questions and developed a new version of Spanish QOL-RA.

The aim of our study was to validate this Spanish version in patients with Rheumatoid Arthritis (RA).

Methods: A cross-sectional multicenter study was carried out. Patients ≥18 years of age, with a diagnosis of RA according to ACR-EULAR 2010 criteria were included. Sociodemographic data, comorbidities, RA characteristics, disease activity current treatment were registered. Questionnaires were administered to assess quality of life by EQ-5D-3L and QOL-RA, functional capacity by HAQ-A and depression by PHQ-9. The time to complete and calculate both quality of life questionnaires was measured, and the difficulties that the patients presented to complete any item were recorded. The QOL-RA was re-administered in 20 patients to evaluate reproducibility. Patients with difficulties in answering the questionnaire (illiterate, blind) and with decompensated comorbidities were excluded. Statistical analysis: Student´s T, ANOVA and Chi2 tests. Spearman correlation. Reliability by Cronbach´s alpha. Reproducibility using ICC. QOL-RA linear tendency based on RA activity by multinomial logistic regression with completed factorial model. Multiple linear regression.

Results: 430 patients were included. Median (m) disease duration was 8.9 years (IQR 4-16). m DAS28-ESR was 2.9 (IQR 1.9-3.9). m QOL-RA was 6.6 (IQR 5.3-8) and it had a good correlation with EQ-5D-3L (Rho: 0.6), PHQ-9 (Rho: -0.56), HAQ-A (Rho: -0.55) and DAS28-ESR (Rho: -0.4). Worse QOL-RA was observed in current smokers (mean 6.1±1.7 vs 6.6±1.9, p= 0.016), unemployed (mean 6.1±1.8 vs 6.9±1.8, p=0.0001) and patients not doing physical activity (mean 6.3±1.9 vs 7±1.9, p=0.0001). It showed very good reliability (0.97) and reproducibility (ICC= 0.96 IC 95% 0.90-0.99). Ceiling and floor effects were 2.8% and 0.7%, respectively. Only 0.9% of the questionnaires presented at least one missing answer. There was no redundancy between questions. Patients with higher disease activity had a significant poorer quality of life. In the multivariate analysis, disability, disease activity, and the presence of depression were independently associated to worse quality of life.

Conclusions: This new version of QOL-RA demonstrated better construct validity, reproducibility and reliability compared to the original version and it was easy to complete and calculate. It it was not influenced by age and disease duration.

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Bogdan Ion Gavrila1, Claudia Ciofu1, Liviu Macovei1, Ioan Ancuta1, Mihai Bojinca1, and Victor Stoica, Prof1. 1Department of Internal Medicine and Rheumatology Cantacuzino Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania.

Objectives: Testing predictive value of 14-3-3 eta protein regarding response to Infliximab (IFX) and its biosimilar (Remsima) on 2 grups of patients diagnosed with rheumatoid arthritis (RA).

Methods: Longitudinal and observational study, including 2 groups of patients with active RA, uncontrolled by csDMARDs, followed 12 months,16 treated with IFX and 17 with Remsima. Clinical assessment was performed at 0,6,12 months according to ACR criteria and evaluation of treatment response according to EULAR criteria (good/moderate/nonresponder).

Results: 30 patients were women and 3 men. Following baseline 14-3-3eta titers and the response at 6 months, tests for identifying differences between groups showed that lower 14-3-3eta protein titers, in both groups of patients, had predictive value on achieving a good EULAR response at 6 months. For IFX, patients with good EULAR response had lower baseline titers(0.11±0.245 ng/ml) than patients with moderate response (0.70±0.705 ng/ml,p=0.049). For Remsima group, good responders had lower baseline titers (0.25±0.380 ng/ml) than nonresponders (1.67±0.615 ng/ml, p=0.004) or those with moderate response (0.25±0.401 ng/ml).

Grouping patients in 2 categories (responders/nonresponders) only for IFX group,14-3-3eta maintained value predicting a 6 months response (0.25±0.378 ng/ml) Vs. the nonresponders (1.67±0.615 ng/ml, p=0.0005).

For 12 months, we did not find significant differences between groups regarding baseline 14-3-3eta titers and the response obtained (IFXp=0.1483, Remsima p=0.2470). The status pretreatment(positive/negative) influenced the good response for IFX group at 6 months(p=0.005).

Regarding the evolution of serum titers, we noticed a reduction, statistically close to significance for IFX (baseline 0.51± 0.703 ng/nl,12months 0.13± 0.439, p=0.064), but not for Remsima(p=0.153).

Conclusions: 14-3-3 eta, a new diagnostic biomarker, could be a major candidate to distinguish pretreatment patients who will or not respond to anti-TNF α therapy.

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Claudia Ciofu1, Liviu Macovei1, Ioan Ancuta1, Mihai Bojinca1, Victor Stoica1, and Bogdan Ion Gavrila1. 1Department of Internal Medicine and Rheumatology, “Dr. I. Cantacuzino” Clinical Hospital, Carol Davila University of Medi, Bucharest, Romania.

Objectives: Evaluating the predictive role for the response to anti-TNF α of cartilage oligomeric matrix protein (COMP), a biomarker which evaluates the articular cartilage degradation and its turnover.

Methods: Observational study including 64 patients followed 12 months with active RA, uncontrolled by csDMARDs. Clinical assessment was performed at 0,6,12 months according to ACR criteria approved by OMERACT and evaluation of treatment response according to EULAR criteria (good/moderate /nonresponder).

Results: 64 patients included. After 6 months,7 patients were declared nonresponders, 38 achieved a moderate response and 19 a good response. Following baseline COMP titers and the EULAR 6-month response, general tests identified significant differences between groups. Lower baseline titers had predictive value for achieving a good response (746.04±130.095 ng/ml) comparing with moderate responders (1032.8±188.671ng/ml) and nonresponders (1042.2±181.717 ng/ml, p=0.000). After 12 months,11 patients achieved moderate response,44 good response and just 1 patient was declared nonresponder. At this visit, even if we did not find significant differences between baseline COMP titers and the EULAR response (p=0.1430), we observed lower baseline titers for good responders (917.8±219.943 ng/ml) vs moderate responders (1042.7±193.117 ng/ml). Grouping patients’ responders/nonresponders there were no differences between groups at 6 months (p=0.227) or 12 months (p=0.9753). Following the status pretreatment of COMP and EULAR response at 6 months, we identified differences between groups (p=0,0001), all 7 nonresponders patients were COMP positive and only 13/19(68.4%) of good responders were tested positive. At 12 months there were no differences between groups (p=0.2805).

Conclusions: COMP could be one of the biomarkers for identifying pretreatment the patients who will respond to anti-TNF therapy in RA.

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Rafaela Santo1,2, Jordana Miranda de Souza Silva1,2, Joshua Baker3, Vanessa Hax1,2, Claiton Viegas Brenol1,2, Lidiane Isabel Filippin4, Priscila Lora5, and Ricardo Machado Xavier1,2. 1 Universidade Federal do Rio Grande Do Sul, Porto Alegre, Brazil, 2 Serviço de Reumatologia do Hospital de Clínicas de Porto Alegre, Porto Alegre, Brazil, 3 University of Pennsylvania, Philadelphia, United States, 4 Universidade La Salle, Canoas, Brazil, 5Universidade do Vale do Rio dos Sinos, São Leopoldo, Brazil.

Objectives: To assess muscle mass relative to fat mass and verify the associations of this parameter with disease activity status, functional capacity and biologics treatments.

Methods: 90 RA patients, aged between 40 and 70 years, were recruited and followed for 12 months. Body composition was assessed by total body dual-energy x-ray absorptiometry for measurement of appendicular lean mass index (ALMI, kg/m2) and fat mass index (FMI, kg/m2). Age-, sex-, and race-specific Z- Scores and T-Scores were determined by comparison to published reference ranges. ALMI values were adjusted for FMI (ALMIZ/FMIZ) using a published method. Disease activity was assessed by Disease Activity Score-28 with erythrocyte sedimentation rate (DAS28). RA patients were divided in non-remission (DAS28>2.6) and in remission (DAS28<2.6). Physical functioning was assessed by the Health Assessment Questionnaire (HAQ). Pharmacological treatment used was assessed from the patient’s medical records; RA patients were divided into RA patients treated with biologic disease modifying antirheumatic drugs (bDMARDs) and non-treated with bDMARDs. Frequency analysis, Pearson Correlations and GEE analyses were used, and statistical significance was considered as p<0.05.

Results: Of the 90 patients analyzed, most were women (86.7%,78/91), with mean age of 56.5±7.3 and median disease duration time of 8.5 (3-18) years. At baseline, the mean±SD DAS28 score was 3.7±1.4 and thirty percent of the RA patients (27/90) were treated with bDMARDs. After 12 months, the use of bDMARD did not change (p>0.05), however, mean DAS28 increased over time (mean and SD of 4.0±1.3; p<0.05). Eleven RA patients (12.2%) showed low ALMI/FMI for age (Z-score ≤-1) at baseline, and 13 (16.0%) after 12 months. After 12 months, ALMIZ/FMIZ was inversely associated with HAQ (r=-0.3; p<0.05). At, baseline, women in remission had higher ALMIZ, lower FMIZ and higher ALMIZ/FMIZ, while men had lower ALMIZ, lower FMIZ and higher ALMIZ/FMIZ. In men, remission was associated with decreases in FMIZ (p<0.05). The use of bDMARDs was not related with alterations in ALMIZ, FMIz and ALMIZ/FMIZ (p>0.05).

Conclusions: Low skeletal muscle mass relative to adiposity was common in RA patients. This condition was associated with low physical functioning and its changes over time are associated with disease activity status. The observations that skeletal muscle mass relative to adiposity was affected by remission state and that it associated negatively with poor physical functioning, demonstrates the importance of adequate control of disease activity in patients with establishedRA. In addition, from our results, further studies are necessary to elucidate the direct impact of bDMARDs on body composition in RA patients.


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Peter Taylor1, Yoshiya Tanaka2, Anabela Cardoso3, Jinglin Zhong4, Yun-fei Chen3, Jennifer Workman3, Liliana del Carmen Morales5, Michael Schiff6, and Diana Gómez5. 1 Botnar Research Centre, Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Sciences, University of Oxford Botnar Research Centre Oxford, Oxford, UK, 2 The First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Kitakyushu, Japan, 3 Eli Lilly and Company, Indianapolis, IN, USA, 4 IQVIA, Morrisville, NC, USA, 5 Instituto Reumatológico Strusberg, Córdoba, Argentina, 6University of Colorado School of Medicine, Denver, CO, USA.

Objectives: The objective of this analysis was to assess if patients (pts) receiving baricitinib (BARI) early gain added clinical improvement compared to pts with delayed start of therapy.

Methods: In RA-BEAM, 1305 pts were randomized 3:3:2 to PBO, BARI-4 mg QD, or adalimumab (ADA) 40 mg every 2 weeks. At Week 24, PBO pts were switched to BARI-4 mg. Patients initially randomized to BARI-4 mg were the early start group and PBO pts rescued at Week 16 or switched at Week 24 or later were considered delayed start. Change from baseline in Clinical Disease Activity Index (CDAI), Simplified Disease Activity Index (SDAI), DAS28-hsCRP, DAS28-ESR, HAQ-DI, and modified total Sharp score (mTSS) were compared between early vs delayed start groups between Weeks 24 and 52.

Results: The early start group had significantly greater change from baseline up to Week 32 with greater and more rapid reduction through first 4 weeks (>50% reduction) for CDAI compared to the delayed start group. At Week 24, the delayed start group showed similar reduction in CDAI as the early start group between Weeks 4 and 8 giving the early start group a 4 to 5-month advantage in disease improvement. After receiving BARI, the delayed start group also showed a rapid improvement in CDAI and caught up to the early start patients by Week 40. Similar results were seen for SDAI, DAS28-ESR, and DAS28-hsCRP. The early start pts maintained significantly greater improvement in HAQ-DI at Week 40 and a significant advantage from Weeks 16 to 52 for mTSS.

Conclusions: While overall disease activity improvement was similar, early start of BARI provided faster efficacy. A delay of up to 6 months in BARI treatment affected HAQ-DI and structural damage progression.

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R. van Vollenhoven1, T. Takeuchi2, A.L. Pangan3, A. Friedman3, M.F. Mohamed3, S. Chen3, M. Rischmueller4, R. Blanco5, R.M. Xavier6, and V. Strand7. 1 Amsterdam Rheumatology and Immunology Center ARC, Amsterdam, the Netherlands, 2 Keio University School of Medicine, Tokyo, Japan, 3 AbbVie Inc, North Chicago, USA, 4 The Queen Elizabeth Hospital and University of Adelaide, Adelaide, Austrailia, 5 Hospital Universitario Marqués de Valdecilla, Cantabria, España, 6 Universidade Federal do Rio Grande do Sul Porto Alegre, Rio Grande do Sul, Brazil, 7Stanford University, Palo Alto, USA.

Objectives: To compare the clinical efficacy, including inhibition of structural damage, and safety of upadacitinib (UPA), a JAK1-selective inhibitor, as monotherapy, vs methotrexate (MTX) monotherapy, in MTX-naïve patients (pts) with moderate to severely active rheumatoid arthritis (RA) at high risk for structural progression.

Methods: In SELECT–EARLY, MTX-naïve pts with active RA who were positive for both RF and ACPA and/or had ≥1 joint erosion was randomized 1:1:1 to once-daily (QD) UPA at 15mg or 30mg, or weekly (wk) MTX (titrated by Wk8). Separate primary endpoints were ACR50 at Wk12 (FDA), or the proportion of pts achieving DAS28CRP <2.6 at Wk24 (EMA). Secondary endpoints included change from baseline (BL) (Δ) in modified Total Sharp Score (mTSS≤0) and proportion of pts with no radiographic progression at Wk24.

Results: 945/947 patients were treated; 88.7% completed Wk24. All primary and secondary endpoints were met. Significantly more patients on UPA15 and 30mg vs MTX achieved Wk12 ACR50 (52.1% and 56.4% vs 28.3%) and Wk24 DAS28CRP <2.6 (48.3% and 50.0% vs 18.5%). At Wk24, mean ΔmTSS was 0.14 and 0.07 vs 0.67 (UPA 15/30 mg vs MTX) and more patients had no radiographic progression on UPA. More patients on UPA vs MTX achieved LDA and remission.

Rate of AEs and SAEs were similar on UPA15 and MTX, and higher on UPA 30. AEs leading to discontinuation were similar across arms. More patients on UPA30 reported serious infections. Herpes zoster was higher on UPA vs MTX. Four malignancies, 4 major adverse cardiovascular events (MACE), and 6 deaths were reported (MTX: 1 sudden [CV]; UPA15: 1 CV, 1 metastatic malignant melanoma; UPA30: 1 CV, 1 pneumonia and sepsis, 1 peritonitis). Two VTE were reported (MTX:1 PE, UPA30:1 DVT).

Conclusions: In MTX-naïve pts at high risk for structural progression, UPA at 15 and 30mg QD demonstrated significant and clinically meaningful improvements in RA signs & symptoms vs MTX. Radiographic progression was significantly lower with UPA vs MTX. Safety events were consistent with Phase 2 and 3 studies with UPA in RA to date.

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Marco Maradiaga Ceceña1, Alan J Kivitz2, Karel Pavelka3, Eva Dokoupilova4,5, Ricardo Blanco6, Hasan Tahir7, Yi Wang8, Brian Porter8, Anna Stefanska9, Susanne Rohrer10, and Hanno Richards10. 1 Centro de Investigación de Tratamientos Innovadores de Sinaloa, Culiacán, México, 2 Altoona Center for Clinical Research, Duncansville, USA, 3 Institute of Rheumatology and Department of Rheumatology, 1st Faculty of Medicine, Charles University in Prague, Prague, Czech Republic, 4 MEDICAL PLUS s.r.o., Uherske Hradiste, Czech Republic, 5 University of Veterinary and Pharmaceutical sciences, Faculty of Pharmacy, Department of Pharmaceutics, Uherske Hradiste, Czech Republic, 6 Division of Rheumatology, Hospital Universitario Marqués de Valdecilla, Santander, España, 7 Barts Health NHS Trust, London, UK, 8 Novartis Pharmaceuticals Corporation, East Hanover, USA, 9 Novartis Ireland Limited, Dublin, Ireland, 10Novartis Pharma AG, Basel, Switzerland.

Objectives: Secukinumab (SEC) provided significant and sustained improvement in the signs and symptoms of ankylosing spondylitis (AS) over 2 years in the MEASURE 3 study (NCT02008916). Here we report 3-year end-of-study results.

Methods: 226 patients (pts) were randomized to intravenous (IV) SEC 10 mg/kg (baseline, Weeks (Wks) 2 and 4) followed by subcutaneous (SC) SEC 300 or 150mg every 4 wks (IV→300/150mg), or matched placebo (PBO). At Wk 16, PBO pts were re-randomized to SC SEC 300 or 150mg. Analysis at Wk 156 included pts initially randomized to SEC and those who switched from PBO to SEC at Wk 16 (Any SEC 300mg, N = 113 and Any SEC 150mg, N = 110). Outcomes at Wk 156 included ASAS20/40, hsCRP, BASDAI, ASAS partial remission (PR) and ASDAS-CRP inactive disease. Analyses stratified by TNF α inhibitor (TNFi)-naïve and TNFi inadequate response [IR] status were pre-specified. Data are reported as observed. Safety analyses included all pts who received ≥1 dose of SEC.

Results: 80.5% (91/113; Any SEC 300mg) and 80.9% (89/110; Any SEC 150mg) pts completed 156 wks of treatment. ASAS20/40 response rates at Wk 156 were 75.0%/ 56.5% in the Any SEC 300mg and 68.2%/47.7% in the Any SEC 150mg groups. Sustained improvements were also observed across all other endpoints through Wk 156. Response rates on more stringent clinical endpoints (eg ASAS40, ASAS PR) were higher with the 300mg dose, particularly in TNFi-IR pts. The safety profile was similar through Wk 156 for both SEC doses. Exposure-adjusted incidence rates for Candida and serious infections were 0.7 and 0.7 per 100 patient-years, respectively, in the combined SEC group over the entire treatment period. No cases of inflammatory bowel disease, including Crohn's disease or ulcerative colitis, were reported, and no deaths occurred.

Conclusions: SEC (300 and 150mg) provided sustained improvements through 3 years in the signs and symptoms of active AS, with greater responses observed with the 300 mg dose. The safety profile of SEC was consistent with previous reports.

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Iain B. McInnes1, Alan J. Kivitz2, Peter Nash3, Proton Rahman4, Jurgen Rech5, Bruce Kirkham6, Sandra Navarra7, Kevin Ding8, Emma Ilsley9, and Luminita Pricop8. 1 University of Glasgow, Glasgow, United Kingdom, 2 Altoona Center for Clinical Research, Duncansville, United States, 3 University of Queensland, Brisbane, Australia, 4 Memorial University, St. John's, Canada, 5 Friedrich-Alexander-University Erlangen-Nürnberg (FAU), Monash University, Erlangen, Germany, 6 Guy's & St Thomas' NHS Foundation Trust, London, United Kingdom, 7 University of Santo Tomas Hospital, Manila, Philippines, 8 Novartis Pharmaceuticals Corporation, East Hanover, United States, 9Novartis Pharma AG, Basel, Switzerland.

Objectives: Secukinumab, a fully human monoclonal antibody that selectively neutralizes IL-17A, provides sustained improvement in the signs and symptoms of active psoriatic arthritis (PsA). 1 Here we report long-term (4-yr) efficacy and safety results in patients from FUTURE 2 study.

Methods: Overall, 397 patients with active PsA were randomized to either secukinumab (300, 150, or 75 mg) or placebo weekly, followed by every 4 weeks (wks) starting at Wk 8.1 Approximately 1/3 of patients had inadequate response [IR] to prior anti-TNF use. Pts were escalated from 150 to 300 mg and from 75 to 150/300 mg starting at Wk 128, if active signs of inadequate control of disease were observed. Assessments at Wk 208 included ACR20/50/70, PASI 75/90, HAQ-DI, SF-36 PCS, and resolution of dactylitis/enthesitis. Analyses by prior anti-TNF use (naïve/IR) and with/without concomitant methotrexate were assessed. Safety analysis included all patients who received ≥1 dose of secukinumab.

Results: Overall, 69/100 (69%), 70/100 (70%), and 62/99 (63%) patients originally randomized to secukinumab 300, 150, and 75 mg, respectively, completed 208 wks of treatment; 46/100 (46%) patients in the 150 mg group were escalated to 300 mg and 56/99 (57%) patients in the 75 mg group escalated to 150/300 mg. Clinical responses were sustained through Wk 208. In the overall population response rates at Wk 208 in the 300, 150, and 75 mg groups were 71.2%, 75.0% and 69.4% for ACR 20, and 80.6%, 81.4% and 66.7% for PASI 75, respectively; the proportion of patients with complete resolution of enthesitis/dactylitis were 70.7%/85.3%, 71.7%/88.0% and 64.4%/92.3%, respectively. In patients who had dose-escalation, the proportion of non-responders and ACR20 responders decreased, with corresponding increases in the proportion of patients achieving ACR50/70 responses. The type, incidence, and severity of adverse events with secukinumab were consistent with previous reports. 1

Conclusions: Secukinumab 300 and 150 mg provided sustained improvement in the signs and symptoms of PsA over 4 yrs. Secukinumab was well-tolerated, with no new/unexpected safety signals.


1 McInnes IB, et al. Rheumatology (Oxford). 2017;56:1993–200.

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José Maximiliano Martínez Pérez1, Dafne Capelusnik2, Carolina Isnardi2, Marina Scolnik1, Gustavo Citera2, and Enrique Soriano1. 1 Hospital Italiano de Buenos Aires, Ciudad Autónoma de Buenos Aires, Argentina, 2Instituto de Rehabilitación Psicofísica, Ciudad Autónoma de Buenos Aires, Argentina.

Objectives: 1) We determined the percentage of patients with PsA and AS who complied with the indication of biological treatment in accordance with international guidelines and the percentage of patients who received it.

2) We identify important gaps for access to biological treatment in these groups of patients and

3) We consider possible solutions to improve access.

Methods: During a two-month period, patients with PsA and AS were evaluated. Compliance with the indication of biological treatment was evaluated according to international clinical guidelines, GRAPPA for PsA and ASAS for AS. The demographic, socioeconomic and clinical characteristics were evaluated. A qualitative analysis (QA) was developed to identify the perception of access barriers to patients. Data on compliance with treatment guidelines were obtained from medical records. In-depth interviews with patients with clear indication of biological products, but not receiving them, were done to better understand patients' beliefs about their disease, how to deal with their disease, their knowledge about their treatment options and their knowledge about access to treatments in general.

Consecutive patients with PsA and AS were evaluated from the outpatient clinics of two rheumatological centers: Hospital Italiano de Buenos Aires (private hospital, PrH) and Institute of Psychophysical Rehabilitation (publichospital, PuH). This study was sponsored by Novartis.

Results: 200 patients were included, 100 with PsA and 100 with AS.

Among all patients with PsA 55 fulfilled GRAPPA criteria to receive biologics, but only 39 of them (71%) received that treatment. There were significative differences between PrH and PuH in the percentage of patients fulfilling criteria. This agrees with patients in the PuH being more active.

Among all patients with AxSpA 57 fulfilled ASAS criteria to receive biologics, but only 35 of them (61%) received that treatment. Only 30% of patients fulfilling criteria in the PuH received biologics, while 93% of those in the PrH received them (p=0.001)

The major reasons for this gap were the refusal to approve the medication by the payer, and the patient’s refusal to receive the new medication, and in AS patients the physician not prescribing it.

Conclusions: In summary there is a gap between criteria for biologics and the actual prescription of biologics. The main reason for not receiving biologics was refusal from the payer and the patient. There were some differences between a private hospital with higher access and a public hospital, and also between PsA and SpA. We still need to work on patient’s education, patients-physicians communication, accessibility, and primary care physicians.

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Clara Malagón1, Maria del Pilar Gómez1, Catalina Mosquera1, Camilo Vargas1, Tatiana González1, Cristine Arango1, Lorena Martin1, and Pilar Pérez1. 1Grupo investigación GRIP. Universidad El Bosque, Bogotá, Colombia.

Objectives: Autoinmune cytopenias are common in pediatric patients. Organ specific and systemic autoimmune diseases (AD) share several pathogenic mechanisms and clinical features that may predispose the co-occurrence of more than one AD within an individual. Polyautoimmunity has been documented on adult and juvenile patients. Multiple autoimmune syndrome is the association of three or more AD. From a registry of 317 patients followed at 15 pediatric rheumatology clinics.

Methods: According to diagnostic criteria, the AD was classified. The index disease was the first AD diagnosed. If the diagnosis was confirmed with an interval less than six months they were classified as simultaneous, if this period was longer, otherwise. The clinical, serologic and the types of associations were determined.

Results: n= 22/317. Female predominance (F22: M1) and wide range of age of onset (1-16 y) were observed in autoinmune hemolytic anemia, autoinmune thrombocytopenia and autoinmune leucopenia. 60% had a positive family history of AD. Al cytopenias were always the index disease and the mean interval between first and second AD was 30,3 but the additional intervals were longer. The only male patient developed AI leucopenia and Hashimoto simultaneously. 3 patients developed bicytopenia during follow up. ITP was the most common index hematological AD (n=18 / 317), followed by Autoimmune leucopenia (3/317) and AI Hemolytic anemia (1/317). Patients with Evans syndrome and cytopenias associated to antiphospholipid antibodies were not included because they were classified as APLS. Multiple associations of AD were documented during follow up.

Conclusions: Autoimmune cytopenias may precede, coincide or follow additional organ specific and systemic AD and even develop MAS. Due to the fact that autoinmune cytopenias may be the index AD in polyautoimmunity, those patients require a close follow up of the clinical course and serologic profile. The intervals between 1° and 2° AD were prolonged. Hematological manifestations are the most common no thrombotic complications of APLS and must be ruled out at onset and during follow up seroconversion of antiphospholipid antibodies has been documented.

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Jenny Anali Yafac Serrano1, Carlos Efrain Huanqui Guerra1, Katherin Paucar Valdivia and Franz Diego Zevallos Zúñiga. 1Hospital Honorio Delgado Espinoza, Arequipa, Perú.

Objectives: To evaluate the efficacy of intraarticular application in the knees of platelet-rich plasma (PRP) plus medical ozone (MO), in patients with rheumatoid arthritis (RA) and clinical knees synovitis refractory to conventional treatment.

Methods: 30 patients with RA and refractory knee synovitis of both sexes were selected for the study. All had definitive diagnosis of RA (DAS 28 ≥2.8) and refractory synovitis of the knees, resistant to conventional RA therapy. The knees were radiographically evaluated, presenting osteoarthritis (OA) grade II to III according to the Kellgren-Lawrence scale.

Treatment consisted of applying 3 cc of PRP and 3 cc of MO at a concentration of 20 μg / ml, in both knees, in 3 doses, once a week.

For the clinical evaluation of knees, visual analogue scale (VAS) was used before, after one month and one year after treatment; in some cases, ultrasonography was also used.

For the comparison of the clinical scores, the VAS scale was used and in the statistical analysis the Student's t test for repeated measurements was used; a p <0.05 was considered as significant.

Results: Of the 30 patients, 1 was a man and 29 were women, with an average age of 58 years. There was clinical improvement in knees according to the VAS scale, with an average of 6.5 at the beginning, 4.86 at one month and 3.43 a year after treatment (p <0.05), figure 1. According to the patients, there was improvement of functional capacity in ambulation in 80% of cases.

There was a decrease in hypertrophy and synovial effusion in all patients (20% of the total) who underwent ultrasonography on their knees one year after treatment.

Conclusions: The application of combination therapy of PRP plus MO in RA patients with refractory synovitis of the knees to conventional medication treatment and physioteraphy, is effective, simple, safe, low cost treatment and can lead to avoid remplacement surgery of the knee.

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Chiara Bertolazzi1, Marwin Gutierrez1, Angelica Vargas2, Tatiana Sofía Rodríguez-Reyna3, Hugo Sandoval1, Everardo Álvarez-Hernández4, Marcelo Audisio5, Eduardo Cabello6, Paola Coral7, Ericka Díaz8, Virginia Duringa, Karinna Espejo9, Selma Gallegos10, Gabriela Hernández-Molina3, Blanca Herrera8, Cristiane Kayser11, María Eugenia Lara12, Genessis Maldonado13, Marta N. Mamani14, Alejandro Nitsche15, Carlos Ríos-Acosta16, Félix Enrique-Romanini14, María Sormani de Fonseca14, Verónica Vilela17, Miguel Villarreal1,8. 1 Instituto Nacional De Rehabilitación, Tlalpan, Mexico, 2 Instituto Nacional de Cardiología Ignacio Chávez, Tlalpan, México, 3 Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Tlalpan, México, 4 Hospital General de México Dr. Eduardo Liceaga, México, 5 Hospital Nacional de Clínicas de la Universidad Nacional de Córdoba, Argentina, 6 Hospital Nacional Alberto Sabogal Sologuren, Perú, 7 Universidad Nacional de Colombia, Colombia, 8 Sociedad Chuquisaqueña de Reumatología, Bolivia, 9 Centro de Excelencia en Reumatología, Perú, 10 Hospital Dr. Darío Fernández Fierro, México, 11 Universidade Federal de São Paulo (UNIFESP), Brazil, 12 Lagomaggiore's Hospital, Argentina, 13 Universidad Espíritu Santo, Ecuador, 14 Hospital Rivadavia, Argentina, 15 Instituto de Rehabilitación Psicofísica, Argentina, 16 Rheumatology and Rehabilitation Center, Ecuador, 17 University Hospital Pedro Ernesto, Brazil, 18Hospital Universitario Dr. José Eleuterio González, México.

Objectives: Introduction: Capillaroscopy (CAP) has progressively gained a central role in daily rheumatology clinical practice thanks to its non-invasiveness, the fact that it is well accepted by patients, and to the flourishing scientific evidence of its relevant role in the diagnosis and assessment of Raynaud´s phenomenon and systemic sclerosis and various rheumatic disorders. However, there are neither guidelines nor consensus for the format and content of the CAP report in rheumatology, which represents an integral and crucial aspect of the CAP examination.

Objectives: The aim of this work was to produce a consensus-based recommendations report for capillaroscopy in rheumatology to be used in daily clinical practice.

Methods: A written Delphi questionnaire regarding capillaroscopy reporting was developed from a literature review and expert consensus. The Delphi questionnaire was sent to 30 rheumatology experts in capillaroscopy around Latin-America (LA), asking them to rate their level of agreement or disagreement with each statement. The exercise consisted of three rounds and a final presential round that took place during the 2018 PANLAR congress held in Buenos Aires, Argentina.

Results: The participants to the first, second, third and presential rounds were 23, 22, 22 and 16, respectively. Fifty-five items were discussed in the first round, 58 in the second, 22 in the third and 10 in the presential meeting. At the end of the exercise, 40 recommendations for capillaroscopy reporting in rheumatology reached consensus.

Conclusions: The PANLAR capillaroscopy study group initiative has produced the first expert consensus for the format and content of CAP reporting in rheumatology. The building of a consensus report in CAP responded to a need expressed by teachers and experts to have a clinical instrument to use in daily practice.

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Luis Carlos Rodriguez Delgado1, Gustavo Leon2, Cristina Reátegui3, Jaime Mendoza1, Jessica Gutiérrez1, Héctor García1, Araceli Bernal1, Denise Clavijo1, Chiara Bertolazzi1, Carlos Pineda1, Marwin Gutierrez1. 1 Instituto Nacional De Rehabilitación, Ciudad De México, México, 2 Hospital Edgardo Rebagliati Martins, Lima, Perú, 3Hospital Guillermo Almenara, Lima, Perú.

Objectives: To describe the ultrasound findings of hands and feet in healthy subjects.

Methods: The study included healthy subjects over 18 years of age without the presence of musculoskeletal symptoms who underwent US examination of hands and feet. In order to evaluate if the US findings are influenced by age, we stratified the subject into two groups: A) younger than 40 yearsand B): older than 40 years. An expert US rheumatologist scanned the dorsal surface of the 32 joints (metacarpophalangeal, proximal interphalangeal, and metatarsophalangeal, 1st to 5th and the wrist bilaterally) and 12 tendons (extensor carpi ulnaris and digital flexors of the hand).

Synovial hypertrophy (SH), synovial effusion (SE), erosions and osteophytes were assessed by both grey-scale (GS) and power Doppler signal (PD). For the scanning technique and definitions, the EULAR/OMERACT were adopted.

Results: A total of 60 healthy subjects were included (40 for group A and 20 for group B). SE was identified in 38 (91%) subjects in group A, and in 20 (100%) in group B; while SH was found in 3 (7.5%) subjects in group A and 18 (90%) in group B (p= 0.001). On the other hand, osteophytes were found in 18 (30%) subjects from group A and in 20 (100%) in group B (p= 0.001). Tenosynovitis was detected in 10 (50%) healthy group B subjects and in 2 (5%) in group A (p= 0.001), in both cases the extensor carpi ulnaris was predominant (14). The PD signal was identified in 2 (1%) subjects in group A and in 4 (1%) for group B. Finally, erosions were found in 3 (7.5%) in group A and in 3 (15%) in group B.

Conclusions: The study showed a wide range of US abnormalities in healthy subjects including SE, SH, even PD which varied according with age. This information is useful for the rheumatologist when performing an US, and for establishing an US threshold abnormality for physiological and pathological variants.

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Consuelo Romero-Sánchez1,2,3, Juliette De Avila3, Andrea Chaparro-Sanabria2, Alejandro Ramos-Casallas3, Lorena Chila-M3, Nataly Delgadillo3, Philippe Chalem-Ch4, Wilson Bautista-Molano2,3, and Juan Manuel Bello-Gualtero1,2. 1 Rheumatology and Immunology Department Hospital Militar Central / Universidad Militar / Universidad El Bosque, Bogotá, Colombia, 2 Clinical Immunology Group-School of Medicine, Universidad Militar Nueva Granada, Bogotá, Colombia, 3 Unit of Oral Basic Investigation, School of Dentistry, Universidad El Bosque, Bogotá, Colombia, 4Fundación Instituto de Reumatología Fernando Chalem, Bogotá, Colombia.

Objectives: Recent advances on the role of the adipokine network in the pathogenesis of Rheumatoid Arthritis (RA) and the possibility of using circulating levels of adipokines as potential biomarkers of disease activity has been described. Similarly, it has been shown that obesity is associated with periodontitis. The mechanisms underlying these associations are not well understood so far, but adipokines may be a pathomechanistic crosslink. Therefore, adipokines may also represent a mechanism whereby periodontal infections can impact on RA. The aim was to establish the association of the adipokines levels with rheumatic activity, Body Mass index (BMI) and periodontal infection.

Methods: A cross sectional study was conducted. 62 patients with early-RA (eRA) according to the ACR/EULAR2010 criteria and 62 healthy controls matched by age and gender were included. A complete medical history was obtained. Adiponectin, adipsin, resistin and vaspin levels were measured using Luminex technology, IL6 and leptin by ELISA. Serum markers such as RA, ESR, CRP, and APCA were evaluated. Disease activity was evaluated by DAS28CRP, DAS28ESR, SDAI and RAPID3.Porphyromonas gingivalis (P. gingivalis) by qPCR. An association analysis was made to evaluate the relationship between adipokines levels, rheumatologic activity, BMI and P. gingivalis using Chi square, Fisher’s Exact or U Mann Whitney test. A logistic regression model was performed to confirm associations. All analyses were performed using IBM-SPSS V25 for Windows with a level of significance of 95%.

Results: In the eRA group, 81.1% were women; their mean age was 48.9±10.9 years; 39.6% were overweight, 13.2% were obese and 85.2% had ACPA>20UI. In the controls 79.6% were women, with a mean age of 48.2±11.1 years; 24.5% were overweight and 6.1% were obese(p=0.028). 39.6% of eRA patients showed moderate activity and 13.2% high activity by DAS28ESR(p=0.001). In eRA, high levels of Leptin were three times more frequent (75.6%) than controls (24.4%) p=0.001; similarly, for adipsin high levels were present in 62.5% of eRA Vs 37.5% in controls, p=0.049. Despite the absence of significant differences for periodontitis among groups it was observed a high frequency of P.gingivalis in eRA 63.5% compared to controls(p=0.004).Patients who showed ACPA>20UI and P.gingivalis simultaneously, exhibited high levels of leptin(OR:8.22;CI95%2.75-24.50;p=0.001), high levels of adipsin(OR:3.06;CI95%1.05-8.97;p=0.041) and DAS28ESR>3.2(OR:2.59;CI95%1.46-4.58;p=0.001). Likewise, patients who concurrently present P. gingivalis, DAS28ESR>3.2, CRP>3.0mg/L and BMI>25 also showed an association with high levels of leptin and adipsin (OR:7.20; CI95%2.6819.33; p=0.0001 and OR:2.69; CI95%1.00-7.28; p=0.005 respectively).

Conclusions: High levels of leptin and adipsin are associated with greater clinical activity in patients with RA in early stages and periodontal infection. These adipokines could be a pathomechanistic link whereby obesity and P gingivalis enhance the risk of clinical activity in eRA.

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Olivia Enríquez-Antonio1, Lilia Andrade-Ortega1, and Fedra Irazoque-Palazuelos1. 1Departamento de Reumatología, CMN 20 de Noviembre, ISSSTE., Ciudad de México, México.

Objectives: The damage score MDI-MYODAM has been recently developed to measure the damage of immune mediated myopathies (IMM). The few publications reporting damage measured by MDI-MYODAM included only Caucasian populations with IMM and found a prevalence of 92%.

There are no studies in Mexican or Latin American populations about the degree of damage related to IMM nor about its correlation with disease activity, association with other comorbidities, duration of disease, type of myopathy, or therapeutic history.

To determine the prevalence of damage measured by MDI-MYODAM in patients with IMM from a Mexican population and the relationship with characteristics of the disease and comorbidities.

Methods: With previously informed consent, we applied both sections of MDI-MYODAM to the cohort of IMM patients of our department. We used formal questionnaires to evaluate demographic variables, characteristics of the disease, activity of the disease (MITAX), therapeutic history, and comorbidities (Charlson index). Descriptive statistics were used with bivariate Spearman, Pearson, or Students t correlations according to the case. p<0.05 was considered as a statistically significant value.

Results: 53 myositis patients were assessed, 75.5% were women. Mean age at diagnosis: 40.7±16.78 years and mean duration of disease: 11.8±8.28 years. 32.07% of patients had polymyositis, 32.07% dermatomyositis, 11.32% juvenile dermatomyositis, 9.43% of patients overlapped with systemic lupus erythematosus, scleroderma or rheumatoid arthritis. Charlson index in total population was 2.72±2.38.

With MDI: 84.9% of the patients had damage, with a score > 0 for at least one category, most in endocrine (54.7%), skeletal (45.2%), or gastrointestinal (37.7%) systems, with a mean total damage score of 3.58±2.87. In MYODAM the mean score for total damage was 6.85±5.06. 13.76% of patients had some degree of disease activity as defined by MITAX.

We found direct correlation between MDI score and chronic use of steroids (r 0.34, 95%CI 0.071-0.567, p<0.014). We could not find association of damage with activity 4.29±4.60 vs no activity 3.47±2.59, p=0.55, nor with the type of myopathy or baseline CK.

Conclusions: Most of our patients with IMM had some type of damage, even from the earliest stages of the disease, with greater impact in endocrine, skeletal and gastrointestinal systems. Patients with chronic or relapsing course tend to have more damage. As expected there was a correlation between damage and comorbidities.

The results of our work indicate that damage increases with the duration of the disease and the use of steroids in a non-linear way.

These results highlight the benefits of a better control of the disease with steroid-sparing treatments.

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Rodolfo Nicolas Alvarado1, Marina Scolnik1, Nicolas Martin Marin Zucaro1, Gelsomina Alle1, María Soledad Trasante Borches1, María Victoria Chiarvetto Peralta1, Luis José Catoggio1, and Enrique Roberto Soriano1. 1Hospital Italiano De Buenos Aires, Ciudad Autónoma De Buenos Aires, Argentina.

Objectives: Cardiac valve involvement in patients with Systemic Sclerosis (SSc) is uncommon, except for tricuspid regurgitation associated with pulmonary hypertension, and data regarding its frequency is inconsistent. Our objective was to estimate the incidence rate of moderate to severe valvular disease in patients with SSc compared with controls.

Methods: We included patients with SSc diagnosis (ACR 2013 criteria) belonging to our health management organization (HMO) and followed by our unit from January 1st 2000 to December 31th 2017. Each patient with SSc was matched by age and sex with 3 to 4 controls of our HMO with at least 1 cardiac ultrasound performed during the study period. Subjects were followed until: a) their death, b) the end of the study, c) they voluntarily left the HMO. Electronic medical records were reviewed, demographic and disease characteristics were collected, and incidence rates of valve involvement were calculated for each valve (moderate/severe valvular disease, valve calcification and valve sclerosis) and compared between SSc and controls.

Results: 127 patients with SSc (108 with limited and 18 with diffuse SSc; 96.1% females; 18.4% Scl-70 positive and 67.2% anti centromere positive) and 497 controls were included. Patients with SSC has a significantly higher incidence of aortic stenosis (1.1 vs 0.3 per 100 patients-year, p < 0.001), mitral regurgitation (1.9 vs 0.9 per 100 patients-year, p = 0.001) and tricuspid regurgitation (1.9 vs 0.4 per 100 patients-year, p < 0.001) than controls. Valvular surgery was significantly more frequent in SSc than controls (0.4 vs 0.1 per 100 patients-year, p = 0.03). Aortic sclerosis (6.7 per 100 patients-year), aortic calcification (2.1 per 100 patients-year), mitral sclerosis (2.3 per 100 patients-year) and mitral calcification (2.9 per 100 patients-year) were also more frequent in SSc patients than controls (p < 0.05 for all comparisons).

Conclusions: We found significant higher incidence rates of aortic stenosis, mitral regurgitation and tricuspid regurgitation in SSc patients compared to their matched controls. Patients with SSc also required more valvular surgery. Moreover, aortic and mitral valves sclerosis and calcification were found more often in SSc patients’ echocardiographies than in controls.

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Hugo Javier Madariaga Charaja1, Maria Elena Luza1, Valery Ascuña1, Carola Cervera1, and Brissette Soto. 1Hospital III Yanahuara Essalud Arequipa Perú.

Objectives: To present safety and efficacy of the concomitant use of Rituximab in patients with autoimmune disease being treated for active tuberculosis (TB)

Methods: We included all patients with active TB and concomitant autoimmune disease treated with Rituximab over the last 5 years, treated in hospital III Yanahuara, Arequipa- Perú.

Results: Five patients with active tuberculosis as well as autoimmune disease were treated with Rituximab at a dose of 1 g, 2 doses with an interval of 15 days every 6 months. 4 Female and 1 male. Median age of 41.6 years. Two with ANCA-associated vasculitis, two with systemic lupus erythematosus and one with rheumatoid arthritis. The most frequent symptoms were fever 60%, cough 60%, dyspnea 40% and thoracic pain 40%. Pulmonary TB was confirmed in 3 patients (BK +), renal TB in 1 patient (BK + in urine) and miliary TB (BK -, miliar type intertitial infiltrate and fever).

Autoimmune disease remained inactive in 100% of patients who received antituberculous treatment associated with the use of rituximab and were cured of active TB infection. There were no adverse events of either treatment.

Conclusions: - Rituximab is apparently effective and safe in patients with active TB associated with rheumatological diseases.

- It could be the treatment of choice in patients with a high risk of TB or a high prevalence of TB.

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Heidi Fernandez1, Andrea Cevallos1, Fernando Naranjo-Saltos1, Ruth Jimbo Sotomayor2, Diego Mera2, Efrain Basantes1, Gabriela Carolina Guevara1, Gina Carrillo1, Alejandra Chacón1, Janeth Diaz1, and Carla Hurtado1. 1 Hospital Eugenio Espejo, Quito, Ecuador, 2Pontificia Universidad Católica del Ecuador, Quito, Ecuador.

Objectives: Determine the prevalence of neuropsychiatric syndromes in a group of patients with systemic lupus erythematosus (SLE) in a hospital in Quito-Ecuador.

Methods: A descriptive, cross sectional study was conducted in 85 patients with SLE in a hospital in Quito-Ecuador. Neuropsychiatric syndromes were evaluated through the use of the MINI International Neuropsychiatric Interview and the Montreal scale. Data were grouped into the 4 most prevalent neuropsychiatric syndromes in patients with SLE according to literature (cognitive impairment, anxiety and mood disorders, and psychosis). There is a known association between the presence of SLE neuropsychiatric manifestations with the antiphospholipid syndrome (APS) and corticosteroid use. A bivariate analysis was used to study the association between these variables.

Results: 85 patients, with an average age of 34.12 ± 11.5 years were included, of which 94% were female. 71% of participants (60 patients) had at least one neuropsychiatric syndrome. The most frequent was cognitive impairment (n = 43, 51%) followed by anxiety disorders (n = 35, 41%), mood disorders (n = 34, 40%) and psychosis (n = 1; 1%). 38% presented mild cognitive impairment and 13% had moderate impairment. No severe cognitive disorders were reported in this study. 38% of participants met criteria for the antiphospholipid (APL) syndrome, and of them, 66% had a neuropsychiatric syndrome (OR = 1.83, p = 0.2). The majority of the patients (n = 84; 99%) were treated with corticosteroids during the study; of them, 59 patients presented neuropsychiatric syndromes (OR = 0.9, p = 0.8). Neither association was statistically significant.

Conclusions: There was a high prevalence of neuropsychiatric syndromes in this cohort of patients. The most prevalent was cognitive impairment, followed by anxiety and mood disorders, similar to other international investigations. The results of this study denote the need to create multidisciplinary units that include rheumatologists, internists, psychiatrists and psychologists for the timely detection and treatment of these manifestations. Additional studies are required to establish risk factors for the development of these syndromes and to evaluate associations with specific APL antibodies.

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Florencia Pierini1, Marina Scolnik1, Valeria Scaglioni1, Javier Rosa1, and Enrique R. Soriano1. 1Hospital Italiano De Buenos Aires, Caba, Argentina.

Objectives: To compare the incidence of osteoporotic fractures in RA patients diagnosed after the year 2000 with matched controls from a university hospital-based health management organization (HMO).

Methods: Consecutive RA patients (n=100) diagnosed after the year 2000 (all fulfilling criteria ACR/EULAR 2010 of AR), from the HMO, were matched (age and sex) with controls (1:2). The follow-up period began at the index date, defined as the date of RA diagnosis for RA patients and the date of the first medical claim at the HMO for the non-RA patients. Subjects were then followed until they voluntarily left the HMO, a fracture occurred, the end of study (May 1st, 2018), or death. Electronic medical records were reviewed and demographic, clinical and treatment data were collected. Incidence rates per 1000 persons-years (PY) of distinct types of fractures after index dates were calculated and compared between groups. A multivariate Cox regression analysis was performed to identify factors associated with fractures.

Results: No difference was found in the overall fracture incidence rate per 1000 PY between RA and controls (19.5, CI 12.7-28.6 vs 12.1, CI 7.7-18.7, p=0.07). In the Cox regression analysis, only age (HR 1.06, 1.02-1.11, p=0.006) and a prior fracture (HR 9.85, 2.97-32.64, p <0.001) were associated with fractures after the index date. Neither a diagnosis of RA (HR 0.86, CI 0.24-3.07, p 0.81) nor a prolonged use (>3 months) of low dose corticosteroids (HR 1.57, CI 0.39-6.23, p 0.52) were associated with increased fracture risk. When analyzing each type of fracture, only vertebral fractures were more common in RA patients compared to controls (12.9 per 1000 PY, CI 8.9-25.8, versus 3.4, CI 1.4-8.1, p=0.01, respectively) but vertebral fractures were not associated with the prolonged use of low dose corticosteroids (HR 3.43, CI 0.74-15.82, p=0.11).

Conclusions: In this cohort of RA patients diagnosed after the year 2000, no overall increased risk of fractures was found in comparison to matched controls. This may be due to a better disease control and to the rational use of corticosteroids.

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Marisel Vanesa Bejarano1, Anastasia Secco1, Marta Mamani1, Romanini Felix1, Natalia Tamborenea1, and Nicolas Lloves Schenone1. 1Hospital Bernardino Rivadavia, CABA, Argentina.

Objectives: To estimate the frequency of traumatic events prior to the diagnosis of RA. To determine the frequency of PTSD in patients with traumatic events prior to the diagnosis of RA. To compare demographic, clinical, serological characteristics, existence of depression and anxiety, according to the presence or absence of PTSD.

Methods: Methods: Observational, analytical, cross-sectional study. Patients with a diagnosis of RA were included according to the ACR Criteria (1987) and ACR EULAR (2010). Patients with another rheumatic or chronic autoimmune disease were excluded. To determine the presence of PTSD, the Traumatic Experiences Questionnaire was used in its Spanish-validated version. To assess the severity of PTSD, the Davidson Trauma Scale was used in its Spanish version. The continuous variables were described as mean and standard deviation (SD) or median and interquartile range (IQR), according to distribution and sample size. The categorical variables were expressed in percentages. For the bivariate analysis of continuous variables we used the Student t or Mann Whitney test, according to the distribution and sample size. For the categorical variables we used Chi square or Fisher's exact test, according to the expected frequency distribution. A logistic regression model was performed taking PTSD as a dependent variable.

Results: 128 patients were included, 86.72% were female, with a mean age of 52.23 years (± 12.39). 55.47% of patients reported at least 1 traumatic event prior to the diagnosis of RA. The most frequently reported traumatic event was the unexpected death of a family member or close friend (39, 50%). 32.39% of the patients who had experienced a traumatic event presented PTSD. 43.48% of patients with PTSD experienced a severe disorder. 63.49% of the patients had some degree of depression and 55.56% of anxiety.

Statistically significant differences were found between the patients who presented PTSD vs those without PTSD in: female sex (100% vs 83.81%, p = 0.04), anxiety (median 12, IQR: 4-18 vs 6, IQR: 0-11 p <0.01) depression (median 14, IQR: 6-19 vs 6 IQR: 0-12 p <0.01).

The main variable that showed an independent association with PTSD was depression (OR: 1.13, 95% CI: 1.05-1.20, p <0.01).

Conclusions: We observed that more than half of patients had experienced at least 1 traumatic event prior to the diagnosis of RA and 32.39% of them presented PTSD.

PTSD was found associated with the female sex, the presence of anxiety and, independently, depression. However, we found no association with the activity or the severity of the disease.

A possible explanation for these findings could be that disease´s characteristics are the result of various factors, with stress being one more determinant, within many existing ones.

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Juan Manuel Sevillano Gutierrez1, Dafne Capelusnik1, Emilce Schneeberger1, and Gustavo Citera1. 1Instituto de Rehabilitación Psicofísica (IREP), CABA, Argentina.

Objectives: To evaluate the survival of MTX treatment, the frequency of adverse events, causes of discontinuation and adherence in patients with RA.

Methods: Consecutive patients 18 years and older with a diagnosis of RA (ACR / EULAR 2010 criteria), who had begun treatment with MTX during their disease were included. Sociodemographic, clinical and therapeutic data were collected by review of the clinical history and personal interviews of patients. Date of initiation and discontinuation of MTX, route of administration, concomitant treatments and consumption of coffee and tobacco were recorded. We analyzed the presence of adverse events (AE), their characteristics and the medical decision about MTX treatment. Adherence to treatment was evaluated using the Compliance Questionnaire Rheumatology questionnaire in its 5-item summary version (CQR5). Statistical Analysis: Descriptive statistics. Chi square test or Fisher's exact test; Student's T-test or Mann Whitney test and ANOVA. Survival of treatment by Kaplan-Meier and comparisons by log Rank. Multiple logistic regression, Cox regression. A p value <0.05 was considered significant.

Results: We included 118 patients, 101 were women (85.6%), with a median age (m) of 56 years (IQR 49-64) and a m disease duration of 10 years (IQR 6-18). Thirty-five patients (29.7%) were smokers and 56 patients (47.5%) consumed coffee. Eighty-five patients (72%) received MTX orally. The m dose of MTX was 15 mg weekly (IQR 15-23.75). 43.2% of patients presented AE associated with MTX, 20 of these patients (16.9%) had to discontinue MTX. Gastrointestinal intolerance was the most frequent AE (27.1%), followed by laboratory test disturbances (12.7%). Level of adherence to treatment was excellent, detecting that 86.6% of the patients presented an adherence ≥80%.

The median cumulative survival of MTX treatment was 348 months (95% CI: 235-460.9). In the univariate and multivariate analyzes, there was no association of survival of MTX with sociodemographic variables, disease characteristics, comorbidities, concomitant treatment, route of administration, coffee consumption nor level of adherence.

Conclusions: In our cohort, adherence and survival of MTX treatment were good. The accumulated survival was almost 30 years. Although there were AE recorded in more than a third of patients, its presence did not determine the discontinuation of treatment in most cases.

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Sofía Fernández2, Rosana Maris Quintana1,2, Stella Orzuza2, Adriana MR Silvestre2, Ana Bensi2, Mario Goñi2, Paola Iglesias2, Nora Mathern2, Vanina García-Bianco2, Romina Nieto1,2, Andrés Gil2, Ana Bouza2, Marcela Valdata2, Bernardo Pons-Estel1,2, and Ingris Pelaez-Ballestas2,3. 1 Centro Regional de Enfermedades Autoinmunes y Reumáticas (GO-CREAR). Rosario, Rosario, Argentina, 2 GLADERPO (Grupo Latinoamericano de Estudios de Enfermedades Reumáticas en Pueblos Originarios), Rosario, Argentina, 3Hospital General de México “Dr. Eduardo Liceaga”, Ciudad de México, México.

Objectives: To describe the experience of social suffering by the indigenous Qom community of patients with RA, and the illness trajectories along with their experience with the local health care system in the city of Rosario, Argentina.

Methods: Qualitative research focus on medical anthropology. Setting: Primary health care. Methodology: ethnographic method, with observations and indepth-interviews. Interviews were recorded and transcribed verbatim. The transcripts were analyzed using an ethnographic approach. A triangulation strategy was implemented for the analysis.

Results: A total of 33 interviews were conducted in 29 individuals with RA. The analysis revealed: 1. “normalization” of their symptoms and of their limitations in performing daily tasks.; 2. The individuals’ relationship with the local health care system was complex and limited in several aspects (e.g. access to health care, continuity of treatment) and; 3. The complexity of help-care seeking process and lack of cultural competence.

Conclusions: RA is a disease that has a negative impact on the daily lives of the Qom people living in Rosario. Improving the relationship between this population and the local health care system as well as the implementation of a RA multidisciplinary approach that takes into account the population cultural sensiivity needs to be a priority.

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Pedro Santos-Moreno1, Fernando Rodriguez1, Laura Villarreal-Peralta1, and Diana Buitrago-Garcia2. 1 Biomab -Centro de Artritis Reumatoide, Bogotá, Colombia, 2SIIES- Investigación y educación en salud, Bogotá, Colombia.

Objectives: Rheumatic and musculoskeletal conditions are a diverse group of diseases that are usually caused by problems of the immune system, inflammation, gradual deterioration of joints, muscles and bones. Rheumatoid arthritis (RA)is considered the most common condition among them; the disease is chronic and might get worse over time. RA is typically painful and limits function causing a major impact on the patients’ quality of life. The objective of this study was to evaluate the quality of life patients with RA using the Quality of life in Rheumatoid Arthritis (RAQol) questionnaire in a specialized RA center in Bogotá, Colombia in 2018.

Methods: We performed a descriptive study, we collected sociodemographic data, the Spanish version of the RAQol was applied; this scale has a score from 1 to 10 where 10 is associated with better quality of life. We excluded patients with psychological or psychiatric disorders. Descriptive epidemiology was performed for each variable’ we performed a comparison of means regarding age and RAQol score.

Results: We interviewed 310 patients. 92% were female and 8% male. Mean age was 60 years, SD 10.5. The average time from diagnosis was 14 years. SD 11. The mean score for the scale was 6.8 ± 1.7. When we evaluated each RAQoL instrument domain. the domain with the highest score was the support from family and friends 7.8 ± 2, follwoed by family interaction and mood. We found a positive association (p<0.05) between age and RAQol score.

Conclusions: Our study showed that our patients have an overall good quality of life. As research has shown (2, 3) the domain related to family and friends support had higher scores and the lowest are related to pain and arthritis. Thus, it is important to develop and to provide multidisciplinary programs in order to improve the patient´s quality of life. On the other hand, further research is needed in order to explore outcomes associated with a better or worse quality of life.

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Laura Coates1, Ana-Maria Orbai2, Valderilio Azevedo3, Joseph C. Cappelleri4, Jade Moser5, Ralph Lippe6, Lihi Eder7, Pascal Richette8, Meng-Yu Weng9, Ruben Queiro Silva10, and Lara Fallon11. 1 University of Oxford, Oxford, United Kingdom, 2 Johns Hopkins University School of Medicine, Baltimore, USA, 3 Universidade Federal do Paraná, Curitiba, Brazil, 4 Pfizer Inc, Groton, USA, 5 The Harris Poll, Rochester, USA, 6 Pfizer Pharma GmbH, Berlin, Germany, 7 Women's College Research Institute, University of Toronto, Toronto, Canada, 8 Lariboisière Hospital, Lariboisière, University of Paris 7, Paris, France, 9 Department of Internal Medicine, Division of Allergy, Immunology and Rheumatology, National Cheng Kung University Medical College and Hospital, Tainan, Taiwan, 10 Rheumatology Division, HUCA, Oviedo, España, 11Pfizer Inc, Montreal, Canada.

Objectives: Psoriatic arthritis (PsA) is a complex disease with high impact on health-related quality of life (HRQoL). The PsA core domain set1 includes pain, patient global assessment, physical function, HRQoL, and fatigue. To evaluate the impact of PsA on health domains, a global survey was developed; we report the results in the context of the PsA Impact of Disease (PsAID) questionnaire due to its high content validity for patients. 2

Methods: A patient-based survey was conducted online from Nov 2, 2017 to Mar 12, 2018. Eligible patients (≥18 years of age) had PsA for >1 year, had visited a rheumatologist/dermatologist in the past 12 months, and had reported using ≥1 synthetic/biologic disease-modifying antirheumatic drug for PsA. Patients reported current symptoms and impact of PsA on daily life. Post-survey, responses were aligned with PsAID health domains. Analyses included descriptive statistics and binomial tests.

Results: 1286 patients/8 countries participated; 84% reported moderate/severe PsA. Social and work PsA impacts were reported by 84% and 81% patients, 56% stopped sports/recreational activities, 42% reported decreased work productivity. Commonly reported major/moderate PsA impacts were on: physical activity (78%), ability to perform activities (76%), emotional/mental well-being (69%). More patients in Brazil, France, Spain, UK reported negative impact on emotional well-being, compared with Australia, Canada, Taiwan (p<0.05). Social impacts included emotional distress (58%), social shame/disapproval (32%), ceased participation in social activities (45%). Most respondents (62%) reported major/moderate impact on work productivity. More patients in Brazil, France, USA reported negative impact on work productivity, compared with Canada, Spain, Taiwan (p<0.05). 97% patients reported musculoskeletal symptoms in the past year: joint pain (79%), tenderness (60%), swelling (60%), stiffness (57%), inflammatory back pain (IBP; 53%). Joint pain (32%) and IBP (12%) were considered the most bothersome; 53% patients taking prescription medication reported joint pain.

Conclusions: All health domains that patients reported on were impacted by PsA in this survey, aligning with the life impact domains of the patient-derived PsAID questionnaire. Most patients reported an impact of PsA on aspects of their social, work, and physical life. These results highlight the impact of PsA on multiple health domains from a patient perspective that should be considered during the shared decision-making processes between healthcare professionals and patients. References: 1. Orbai AM et al. Ann Rheum Dis 2017; 76: 673–80.

2. Gossec L et al. Ann Rheum Dis 2014; 73: 1012–9.

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Proton Rahman1, Regan Arendse2, Isabelle Fortin3, Andrew Chow4, Majed Khraishi5, Suneil Kapur6, Michel Zummer7, Raheem Kherani8, Emmanouil Rampakakis9, Odalis Asin-Milan10, Allen J. Lehman10, and Francois Nantel10. 1 Memorial University, St John's, Canada, 2 University of Saskatchewan, Saskatoon, Canada, 3 Centre de Rhumatologie De l’Est du Quebec, Rimouski, Canada, 4 Credit Valley Rheumatology, Missisauga, Canada, 5 Nexus Clinical Research, St John's, Canada, 6 Private practce, Ottawa, Canada, 7 Hopital Maisonneuve-Rosemont, Montreal, Canada, 8 University of British Columbia, Vancouver, Canada, 9 JSS Medical Research, Montreal, Canada, 10Janssen Inc., Toronto, Canada.

Objectives: To describe the profile of psoriatic arthritis (PsA) patients selected for treatment with infliximab (IFX), golimumab (GLM) or ustekinumab (UST) treatment in Canadian routine care and to describe the long-term real-world effectiveness and safety of these agents.

Methods: 462 PsA patients treated with IFX, GLM or UST were enrolled into the Biologic Treatment Registry Across Canada (BioTRAC) registry between 2006-2015, 2010-2017 and 2014-2017, respectively. Study visits occurred at baseline and every 6 months thereafter. Effectiveness was assessed with changes in TJC28, SJC28, skin, enthesitis, dactylitis, pain, HAQ, acute phase reactants. Safety was evaluated with the incidence of adverse events (AEs) and drug survival rates.

Of the 111 IFX-, 281 GLM- and 70 UST-treated patients, the proportion of males were 52.3%, 46.3% and 37.1%, the mean age was 48.4, 52.8 and 53.1 years and the mean disease duration was 5.8, 6.1 and 5.7 years, respectively. Most patients were bio-naive (85.6%, 77.9% and 55.7% for IFX, GLM and UST, respectively (p<0.001). UST-treated patients had lower baseline DAS28 CRP (3.4 vs 3.9; p=0.0031), SJC (3.8 vs 5.3; p=0.0046) and higher PASI (4.8 vs 2.2; p=0.0061) compared to patients treated with GLM.

Results: Treatment with IFX, GLM and UST was associated with significant improvements in all disease parameters over time (p<0.001), respectively with similar efficacy between agents. The only exception was the proportion of patients in minimal disease activity at 12, 24 and 36 months which reached 40.7%, 50.0% and 55% in IFX-patients; 64.7%, 68.8% and 78.9% in GLM-patients and 58.8%, 60.0% and 83.3% in UST-patients (p=0.004 and p<0.001 vs IFX).

AEs were reported for 74.8%, 69.8% and 52.9% (138, 114 and 115 events/100 PYs) and SAEs for 19.8%, 8.5% and 5.7% (8.8, 19.6 and 28.6 events/100 PYs) covering 325, 567 and 87 years of exposure for IFX-, GLM- and UST-treated patients, respectively. One, one and no deaths occurred IFX-, GLM- and UST-treated patients, respectively. The proportion of patients who discontinued treatment were 63.1%, 50.9% and 50.0% over a mean exposure of 2.9, 1.9 and 1.2 years to IFX, GLM and UST, respectively.

Conclusions: Differences in baseline characteristics between patients treated with an anti-TNF over an anti-IL12/23 agent suggest that the level of joint to skin involvement might be driving physician choice when the time comes to choose a biologic agent. IFX, GLM and UST treatment significantly reduced disease activity and improved functionality in a similar fashion and were well tolerated in patients with PsA.

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Philip Mease1, Désirée van der Heijde2, Robert Landewé3, Shephard Mpofu4, Proton Rahman5, Hasan Tahir6, Atul Singhal7, Elke Böttcher8, Sandra Navarra9, Xuan Zhu10, Aimee Readie10, Ken Abrams10, and Luminita Pricop10. 1 Swedish Medical Center and University of Washington, Seattle, United States, 2 Leiden University Medical Centre, Leiden, Netherlands, 3 University of Amsterdam, Amsterdam, Netherlands, 4 Novartis Pharma AG, Basel, Switzerland, 5 Memorial University of Newfoundland and Labrador, St. John's, Canada, 6 Whipps Cross University Hospital, Barts Health NHS Trust,United Kingdom, 7 Southwest Rheumatology, Dallas, United States, 8 Rheumazentrum Favoriten, Vienna, Austria, 9 University of Santo Tomas Hospital, Philippines, 10Novartis Pharmaceuticals Corporation, East Hanover, United States.

Objectives: Secukinumab (SEC), a fully human anti-interleukin-17A mAb, significantly improved signs and symptoms and inhibited radiographic progression versus placebo (PBO) at Week (Wk) 24 in patients (pts) with psoriatic arthritis (PsA) in the FUTURE 5 study. 1 Here, we report the effects of SEC on radiographic progression, additional efficacy endpoints and safety through 52 wks.

Methods: Pts (N=996) with active PsA, were randomized (2:2:2:3) to subcutaneous (sc) SEC 300mg with loading dosage (LD; n=222), 150mg with LD (n=220), 150mg without LD (n=222), or PBO (n=332). All groups received SEC or PBO at baseline (BL), Wks 1, 2, 3, and 4, and then every 4 wks. At Wk 16, PBO non-responders were switched to SEC 300mg or 150mg; remaining PBO pts were switched at Wk 24. Radiographic progression was assessed by mean change in van der Heijde-modified total Sharp score for PsA (mTSS). Pts were stratified based on anti-TNF status (naïve/inadequate response [IR]). Additionally, radiographic data were analyzed by linear mixed effects model (random slope) at Wk 24 and 52. Efficacy assessments at Wk 52 included ACR20/50, PASI75 and resolution of dactylitis and enthesitis. Safety analyses included all pts who received ≥1 dose of SEC.

Results: 91.9% (204/222; 300mg LD), 91.4% (201/220; 150mg LD) and 86.9% (193/222; 150mg No LD) pts completed 52 wks of treatment. Inhibition of radiographic progression was sustained through 52 Wks. Proportions of pts with no radiographic progression (change from BL in mTSS ≤0.5) with SEC at 52 Wks were 92% (300 mg LD), 85% (150 mg LD), and 87% (150 mg No LD). Mean changes from baseline in mTSS by linear mixed effects model at Wk 24 were 0.03 (P<0.01), 0.14 (P<0.05) and -0.10 (P<0.001) respectively, vs. 0.51 (PBO). Corresponding mean change at Wk 52, was -0.18 (300mg LD), 0.11 (150mg LD) and -0.20 (150mg no LD). ACR20/50 responses at Wk 52 were, 68.9/46.8, 64.1/41.4 and 65.8/43.2 in the 300mg LD, 150mg LD and 150mg no LD groups, respectively. Other clinical responses were sustained or improved through 52 wks. No new or unexpected safety signals were identified.

Conclusions: Secukinumab provided sustained inhibition of radiographic progression through 52 wks. Clinical responses were also sustained through 52 wks. The safety profile was consistent with that previously reported. 1

References: 1. Ann Rheum Dis. 2018;77:890-897.

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J. Smolen1, P. Bergmans2, I. Bondareva3, K. de Vlam4, E. Gremese5, B. Joven-Ibáñez6, T.V. Korotaeva7, M.T. Nurmohamed8,9, P.P. Sfikakis10, S. Siebert11, P. Smirnov12, E. Theander13, G. Valentini14, and L. Gossec15,16. 1 Medical University of Vienna, Vienna, Austria, 2 Biometrics, Janssen-Cilag B.V., Breda, Netherlands, 3 Kemerovo Regional Clinical Hospital, Kemerovo, Russia, 4 University Hospitals Leuven, Leuven, Belgium, 5 Fondazione Policlinico Universitario Gemelli - Università Cattolica del Sacro Cuore, Rome, italy, 6 Hospital Universitario 12 de Octubre, Madrid, España, 7 Nasonova Rheumatology Research Institute, Moscow, Russia, 8 VU University Medical Centre, Amsterdam, Netherlands, 9 Amsterdam Rheumatology and Immunology CVU University Medical Centre, Amsterdam, Netherlands, 10 1st Dept. of Propaedeutic Internal Medicine, Joint Academic Rheumatology Program, Athens University, Athens, Greece, 11 Institute of Infection, Immunity and Inflammation, University of Glasgow, Glasgow, United Kingdom, 12 Biometrics, Janssen-Cilag B.V., Moscow, Russia, 13 Biometrics, Janssen-Cilag B.V., Solna, Sweden, 14 Università della Campania “Luigi Vanvitelli”, Naples, Italy, 15 Hôpital Pitié Salpêtrière, Paris, France, 16Sorbonne Université, Paris, France.

Objectives: The purpose of PsABio (ClinicalTrials.gov ID: NCT02627768) is to evaluate the persistence, therapy regimens, efficacy and tolerability of 1st, 2nd and 3rd-line biological disease-modifying antirheumatic drugs (bDMARDs) – tumor necrosis factor inhibitors (TNFi) and ustekinumab (UST) – in psoriatic arthritis (PsA). Here we present first follow-up data on joint-related outcomes.

Methods: Of 278 UST-treated and 285 TNFi-treated patients, enrolled up to August 2017, 152 and 151 patients respectively had reached the 6-month follow-up while remaining on the initial treatment line. We present joint-related outcomes as observed data, comparing baseline to follow-up within the cohorts. As baseline characteristics show differences in factors potentially influencing treatment effects, after full enrollment, between-group comparisons were adjusted using propensity score analysis.

Results: Of 303 patients: mean (SD) age=49.7 (12.8) years; disease duration=7.2 (8.2) years; 50.5% female. At 6 months’ follow-up, 7.6% UST-treated and 10.2% TNFi-treated patients stopped/switched bDMARD. Line of treatment differed (UST: 40%, 36%, 24%; TNFi: 64%, 29%, 7.3% for 1st, 2nd, 3rd-line respectively). Prevalence of concomitant sDMARD differed (UST: 40% [29% MTX]; TNFi: 54% [40% MTX]). DAS-28 improved significantly from baseline, UST: 4.3 (SD 1.2), TNFi: 4.3 (SD 1.3) by mean (95%CI) -1.3 (-1.6, -1.0) and -1.3 (-1.5, -1.2). Significant improvements seen in both cohorts across all treatment lines and PsA subtypes (data not shown). CDAI also significantly improved, by mean (95%CI) -10.9 (-13.1, -8.6) and -10.8 (-12.6, -9.0) in UST and TNFi cohorts: 14% and 15.5% reaching CDAI remission. For DAPSA, statistically significant improvements, mean (95%CI) UST: -18.4 (-22.2, -14.5) and TNFi: -19.5 (-22.5, -16.5). cDAPSA also significantly improved in both cohorts: mean (95%CI) -15.6 (-18.6, -12.5) and -16.9 (-19.4, -14.4) for UST and TNFi, respectively.

Conclusions: Both UST-treated and TNFi-treated patients showed statistically significant and considerable improvements in joint-related measures after 6 months in a real-world setting, irrespective of line of treatment and subtype of psoriatic disease.

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Alberto Spindler1, Nicola Ruperto2, Cesar Francisco Pacheco Tena3, Ingrid Louw4, Gabriel Vega-Cornejo5, Daniel Kingsbury6, Michael Clark7, Karen Bensley7, Xiaoming Li7, and Hermine Brunner8. 1 Centro Médico Privado de Reumatología, San Miguel de Tucuman, Argentina, 2 Istituto Giannina Gaslini, Pediatria II, Reumatologia, PRINTO Coordinating Centre, Genoa, Italy, 3 Universidad Autónoma de Chihuahua, Circuito Universitario Campus II, Chihuahua, Mexico, 4 Panorama Medical Centre, Cape Town, South Africa, 5 Hospital México Americano, Guadalajara, Mexico, 6 Randall Children's Hospital at Legacy Emanuel, Portland, United States, 7 Janssen Research & Development, LLC, Spring House, United States, 8Cincinnati Children’s Hospital Medical Center, Cincinnati, United States.

Objectives: To assess efficacy & safety of intravenous golimumab in pediatric patients with active polyarticular course juvenile idiopathic arthritis despite methotrexate therapy through 28 weeks of treatment.

Methods: A multicenter, Phase 3, single arm, open-label trial was conducted using intravenous golimumab at a dose of 80mg/m2 given at weeks 0 & 4, then every 8 weeks thereafter, in pediatric patients ages 2-17 years of age with active polyarticular course juvenile idiopathic arthritis despite methotrexate therapy. Patients received commercial MTX weekly at same BSA-based dose as at time of study entry. All the results below are based on full analysis set which includes all patients who received at least 1 dose of study agent.

Results: 180 patients were screened (130 enrolled,127 treated) with the first patient screened on 22Dec2014; last patient first treated 26Dec2017, & last patient’s Wk28 visit was 09Jul2018.

Proportion of JIA ACR 30,50,70, & 90 responders at Wk28 was 83.5%,79.5%,70.1%, & 46.5%, respectively. 29.1% of patients met criteria for inactive disease at Wk28. Median change from baseline for JASDAS 10, 27, & 71 was -14.20, -16.60, & -20.32, respectively at Wk28. JADAS 10, 27, & 71 minimal disease activity was met by 15% of patients at Wk28.

Proportion of patients experiencing at least 1 treatment-emergent AE through Wk28 was 77.2%. MedDRA system organ class with highest incidence of AEs was Infections & infestations (57.5%); most commonly reported AE upper respiratory tract infection (17.3%), then nasopharyngitis (15.0%). Six patients experienced serious AEs through Wk28: Herpes zoster disseminated, Infective exacerbation of bronchiectasis, Sepsis, Varicella, Mycosis fungoides, & Suicidal ideation. These events resulted in permanent discontinuation of intravenous golimumab, except for Varicella.

Conclusions: Intravenous golimumab delivered at a dose of 80mg/m2 at weeks 0 & 4, then every 8 weeks thereafter appears to be effective in these patients with a safety profile similar to other TNF inhibitor therapies.

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Clara Malagón1, Maria del Pilar Gómez1, Catalina Mosquera1, Camilo Vargas1, Tatiana González1, Cristine Arango1, Lorena Martin1, and Pilar Pérez1. 1Universidad El Bosque, Bogotá, Colombia.

Objectives: Organ specific and systemic autoimmune diseases (AD) share several pathogenic mechanisms and clinical features that may predispose the co-occurrence of more than one AD within an individual. Polyautoimmunity has been documented on adult and juvenile patients. Multiple autoimmune syndrome is the association of three or more AD. From a registry of 317 patients followed at 15 pediatric rheumatology clinics, 6 patients developed one or more dermatologic AD during the follow up.

Methods: According to diagnostic criteria and/or skin biopsy the AD was classified. As index disease was the first diagnosed AD. If the diagnosis was confirmed with an interval less than six months they were classified as simultaneous if this period was longer. The clinical, serologic and the types of associations were determined.

Results: Vitíligo, Localized scleroderma, Urticaria Vasculitis, psoriasis and additional lupus were identified as skin AD. Female predominance and wide range of age of onset were observed in all of them. They were the index AD disease, second, third or even fourth AD. As index disease Vitiligo was the most common dermatological AD (n= 17/ 317), followed by localized scleroderma (9/317), discoid lupus (5/317) urticaria vasculitis (5/317) psoriasis (2/317) and alopecia universalis (2/317). Multiple associations of AD were documented during follow up. Well recognized associations were identified but unusual clusters were also confirmed. The most common associations were: Vitíligo and Hashimoto, Sclerodermia and Vitíligo but associations with systemic AD were also frenquent. Patients that developed MAS developed one or two skin AD at any time of the follow up and one boy developed scleroderma, psoriasis and vitiligo sequentially but not any systemic AD.

Conclusions: Dermatological ADs may precede, coincide or follow addional organ specific and systemic AD and become as component of SAM. Patients with these diseases should be followed because of the risk of additional organ specific and systemic AD. A variable but significant percentage of cases developed MAS. Changes in the clinical course and/or serologic profile must alert the clinician about polyautoimmunity.

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John Wallace1, Andre Buret1, David Vaughan2, Marcelo Muscara4, Gilberto De Nucci5, Peter Nagy. 1 University of Calgary, Calgary, Canada, 2 University of Calgary, Calgary, Canada, 3 Antibe Therapeutics Inc., Toronto, Canada, 4 University of Sao Paolo, Sao Paulo, Brazil, 5 University of Campinas, Campinas, Brazil, 6National Institute of Oncology, Budapest, Hungary.

Objectives: Hydrogen sulfide (H2S) is a naturally occurring gaseous mediator produced by various eukaryotic cells and by intestinal bacteria. It can exert cytoprotective and analgesic effects. ATB-346 is an H2S-releasing derivative of naproxen that was found to be more potent and long-acting than naproxen. In animal studies, ATB-346 produced negligible gastrointestinal (GI) damage and bleeding, despite profound inhibition of cyclo-oxygenase (COX). A human efficacy study demonstrated that ATB-346 (250 mg daily) was very effective in reducing pain in patients with osteoarthritis of the knee, and inhibiting COX activity. This study tested the hypothesis that ATB-346 is GI safer than naproxen.

Methods: This was a double-blind, active control study. 244 healthy volunteers completed the study. Upper GI endoscopy was performed prior to and on day 14 after commencing treatment with naproxen (550 mg twice daily) or ATB-346 (250 mg once daily in the morning and placebo once daily in the evening). Whole blood thromboxane synthesis (COX activity) and plasma hydrogen sulfide levels were also measured.

Results: For the primary endpoint, incidence of ulcers ≥3 mm in diameter, 53 subjects (42%) taking naproxen developed at least one ulcer, while only 3 subjects taking ATB-346 developed at least one ulcer (p<0.00001). The two drugs produced comparable suppression of systemic COX activity. Subjects in the naproxen group developed more ulcers per subject (an average of 4) than in the ATB-346 group (1.3/subject), and a greater incidence of larger (≥5 mm diameter) ulcers (125 vs. 0), respectively. The incidence of abdominal pain, gastro-esophageal reflux and nausea were markedly lower with ATB-346 than with naproxen. Systemic COX activity was inhibited by 95% in both groups, and plasma H2S levels were significantly elevated in subjects treated with ATB-346 (by ~50%; p<0.001).

Conclusions: As in pre-clinical studies, this phase 2 clinical trial demonstrated a dramatic increase in the GI safety of ATB-346 versus one of the most commonly used NSAIDs, naproxen. ATB-346 produced equivalent suppression of COX to naproxen, consistent with a previous Phase 2A clinical trial that demonstrated significant pain relief in patients with osteoarthritis of the knee. ATB-346 appear to be an effective and much safer alternative to existing NSAIDs.

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Yusuf Yazici1, Timothy McAlindon2, Allan Gibofsky3, Nancy Lane4, Christian Latterman5, Nebojsa Skrepnik6, Chris Swearingen1, Anita DiFrancesco1, Jeyanesh Tambiah1, Marc Hochberg7, and Mirta Grifman1. 1 Samumed, LLC, San Diego, United States, 2 Tufts Medical Center, Boston, United States, 3 Weill Cornell Medical College and Hospital for Special Surgery, New York, United States, 4 UC Davis Medical Center, Davis, United States, 5 Brigham and Women’s Hospital, Cambridge, United States, 6 Tucson Orthopedic Institute, Tucson, United States, 7University of Maryland School of Medicine, Baltimore, United States.

Objectives: A phase 2a study of SM04690, a small-molecule, intra-articular (IA) Wnt pathway inhibitor reduced knee pain and improved physical function and medial joint space width (mJSW) at 52 weeks in subgroups of subjects with unilateral symptomatic knee osteoarthritis (OA) compared to placebo (PBO).1

A 24-week phase 2b study was conducted to refine patient reported outcome (PRO) measures, target population, medication dose, and to evaluate safety. PRO results for Weeks 12 and 24 are presented here.

Methods: Study subject inclusion criteria required ACR-defined knee OA, Kellgren-Lawrence (KL) grade 2 or 3, and Pain Numeric Rating Scale (NRS) scores ≥4 and ≤8 in the target knee and <4 in the contralateral knee. A single IA injection of 2 mL SM04690 (0.03, 0.07, 0.15, or 0.23 mg), vehicle PBO, or sham (dry needle only) was given in the target knee at baseline. PRO endpoints included change from baseline in weekly average of daily pain in the target knee by NRS diary (NRS) [0-10], Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC) Pain [0-100], WOMAC Physical Function [0-100], and Patient Global Assessment (PTGA) [0-100]. Differences between active treatment groups and vehicle PBO were analyzed with baseline-adjusted analysis of covariance (ANCOVA).

Results: 695 subjects (mean age 59.0 [±8.5] years, BMI 29.0 [±4.0] kg/m2, female 58.4%, KL3 57.3%) were enrolled and dosed; 635 subjects (91.4%) completed the study. No meaningful differences in the incidence of adverse events were observed between treatment and control groups.

In the Full Analysis Set, significant improvements from baseline compared to vehicle PBO were observed in pain NRS for 0.07 mg (Week 12 [P=0.001], Week 24 [P=0.031]) and 0.23 mg (Week 12 [P=0.012], Week 24 [P=0.022]) SM04690 dose groups. Similar improvements were observed in WOMAC Pain for 0.07 mg (Week 12 [P=0.04]) and 0.23 mg (Week 12 [P=0.003], Week 24 [P=0.031]) dose groups. For WOMAC Physical Function, improvements were observed for 0.07 mg (Week 12 [p=0.021]) and 0.23 mg (Week 12 [p=0.006], Week 24 [P=0.017]) dose groups. PTGA improvements were observed for 0.07 mg (Week 12 [P=0.031]) and 0.23 mg (Week 12 [P=0.010], Week 24 [P=0.033]) dose groups.

Conclusions: SM04690, in development as a potential disease-modifying OA drug, showed in this Phase 2b study statistically significant improvements from baseline in both the 0.07 mg and 0.23 mg dose groups compared to vehicle PBO for Pain NRS, WOMAC Pain, WOMAC Physical Function, and PTGA. These data support the continued development of SM04690 as a treatment for knee OA. Phase 3 studies are being planned.

References 1. Yazici Y, et al. Arthritis Rheumatol. 2017; 69 (suppl 10).

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Vicente Aldasoro Cáceres1, Javier Mendizábal Mateos1, Sara García Pérez1, Inmaculada Paniagua Zudaire1, Concepción Fito Manteca1, Loreto Horcada Rubio1, Natividad Del Val Del Amo1, Ricardo Gutiérrez Polo1, Laura Garrido Courel1, Juliana Restrepo Vélez1, Rosario Ibáñez Bosch1, and Eduardo Loza Cortina1. 1Complejo Hospitalario De Navarra, Pamplona, España.

Objectives: Tocilizumab (TCZ) has shown efficacy in clinical trials and in real-world data on giant cell arteritis (GCA). We describe our experience with TCZ in GCA in those patients with inefficacy to previous therapies in a tertiary hospital.

Methods: Search and review clinical data of patients diagnosed with GCA and treated with TCZ in the last 5 years.

Results: 23 patients (3 men); mean age 71.5 (7.8). 57% positive temporal artery biopsy. 74% received treatment with sDMARD. 26% started TCZ in combination with methotrexate. Before TCZ was started, the median methotrexate and steroids doses were 20 mg (10-23.75) and 12.5 mg (5-30), respectively. 5 patients had visual lost: 2 amaurosis and 3 optic neuritis.

TCZ was started after a median time from GCA diagnosis of 12 months (2-21). Symptoms in all patients but one improved totally or partially; their CRP an ESR decreased to normal levels at month 1 (p<0.0001). 11/15 patients were asymptomatic at one year. 74% TCZ intravenously. We did not find different efficacy related to the administration route. 3 patients stopped TCZ: respiratory infection, lost of efficacy and hypertransaminasemia at 24, 18 and 2 months, respectively.

8 patients developed toxicity to steroids: 3 diabetes, 3 behavior alterations and 2 myopathies. Most relevant adverse side-effect was serious infections observed in 6/23.

Conclusions: In our series TCZ was effective, fast and safe treatment in the management of GCA refractory to other treatments.

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Miguel Antonio Mesa Navas1,2, Andrea Restrepo Acosta2, Carlos Jaime Velásquez Franco1,2, Libia Maria Rodriguez Padilla2, and Laura Escobar2. 1 Clínica Universitaria Bolivariana, Medellín, Colombia, 2Escuela de Ciencias de la Salud, Universidad Pontificia Bolivariana, Medellín, Colombia.

Objectives: To evaluate the impact of a capillaroscopy training on non-expert professionals and its correlation with experience capillaroscopists in Medellín Colombia, 2017.

Methods: This study was quasi-experimental in its design and included individuals older than 18 who were medics or medical students and gave consent. Participants received a 30-minute introduction and were evaluated (time 1) with a test in which 60 photos were displayed for 30 seconds each. Participants were asked to assess the shape, size and general pattern of nail fold capillaries (20 photos in each section). Subsequently, a six-hour training was carried out, and a new evaluation was performed (time 2) with the same images as in time 1 but in a different order. The agreement among experts was considered the gold standard. The concordance between the experts (inter-reader, intra-reader) and the agreement of each participant with the expert at the two times were evaluated by the Kappa coefficient. The results obtained between the two times were compared using the Wilcoxon test.

Results: 56 medical professionals and two experts were included. The inter-reader and intra-reader agreement between the experts were substantial (Kappa between 0.61-0.80) and almost perfect (Kappa between 0.81-1.00), respectively. After the training, a significant increase was observed both in the number and in the percentage of correct answers with respect to the consensus of experts (p <0.001). The kappa coefficient for time 1 was: 0.50 (CI 0.33-0.71) for form, 0.76 (CI 0.53-0.79) for size, 0.45 (CI 0.29-0.57) for general pattern and 0.71 (CI 0.59-0.84) recognizing specifically scleroderma pattern. At time 2 the kappa was 0,80 (CI 0,60-0,90) for form, 0,76 (CI 0,55-0,80) for size, 0,64 (CI 0,46-0,79) for general pattern and 1 (CI: 1-1) for normal pattern vs systemic sclerosis.

Conclusions: A short Nailfold videocapillaroscopy training can improve the ability of non-expert personnel to identify capillaroscopic abnormalities. This is particularly important when recognizing the scleroderma pattern where the agreement with the gold standard was perfect.

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Denise Clavijo Cornejo1, Aiana Francisco-Balderas2, Alberto A. López-Reyes1, Jose Peralta-Aguilar3, Javier Fernández Torres1, Yessica Zamudio-Cuevas1, Luis Silveira-Torre4, Janitzia Vazquez-Mellado5, and Gabriela Angelica Martínez-Nava1. 1 Instituto Nacional De Rehabilitación, Ciudad de México, México, 2 Instituto Politecnico Nacional, Ciudad de México, Mexico, 3 Universidad de la Salle, Ciudad de México, México, 4 Instituto Nacional de Cardiología, Ciudad de México, México, 5Hospital General de México Eduardo Liceaga, Ciudad de México, México.

Objectives: Gout is caused by the deposition of monosodium urate crystals mainly in joints; in Mexico, the prevalence of gout is estimated at 0.3% but real data are still unknown. Gout is a disabling chronic disease resulting from a high serum uric acid (UA) levels. It is known that renal urate excretion is involved in the development of the disease; studies at the genetic level have shown that single nucleotide polymorphisms (SNP) are related with gout. The aim of this study was to analyze the association between the SNPs of uric acid transporters’ genes and gout susceptibility in Mexican subjects.

Methods: 62 patients with gout (ACR/EULAR criteria), and 165 healthy subjects age ≥18 years old were included. Eight SNP of seven different genes and 11 ancestry-informative markers were genotyped using TaqMan probes in a Quant Studio 12kFlex instrument. The allelic discrimination and ancestry analysis were carried out using the TaqMan genotyper and STRUCTURE1.2.1 software, respectively. For the associations between the genetic variants and gout the odds ratios (ORs) with 95% confidence intervals were calculated by multinomial logistic regression models.

Results: The ABCG2 rs2231142 (T allele) and BCAS3 rs2079742 (C allele) minor alleles were associated positively with gout (OR=3.48, p<0.01 and OR= 3.02, p<0.01; respectively). On the other hand, a significantly lower risk for gout was found in the carriers of the minor allele of ATXN2 rs653178 (C allele OR=0.44 p=0.047) and SFMBT1 rs6770152 (G allele OR=0.42 p<0.01) polymorphisms. Notwithstanding we did not observe a significant association between gout diagnosis and the minor allele of GCKR (rs780094 and rs1260326), OVOL (rs642803) and PRKG2 (rs10033237) polymorphisms; these showed a significant association with the disease when analyzed by genotype in a co-dominant inheritance model.

Conclusions: In a Mexican population, the prevalence and incidence of gout is underestimated; the disease may be chronic and disabling. It is necessary to carry out studies in order to identify the genetic susceptibility to the disease. This preliminary study suggest that the SNPs present in the uric acid transporters can be used as susceptibility markers in Mexican population; however, we are aware that larger sample size studies are required. Financed by FOSSIS-2016; Project-273143.

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Jorge Rojas-Serrano1, José Guillermo Mejía-Hurtado1, Montserrat Ixchel González-Perez1, Enrique Ambrocio-Ortiz2, Ramcés Falfán-Valencia2, Gloria Pérez-Rubio2, Heidegger Mateos-Toledo1, Mayra Mejia1, and Ivette Buendía-Roldán3. 1 Unidad de Enfermedades del Intersticio Pulmonar y Reumatología, Instituto Nacional De Enfermedades Respiratorias. Ismael Cosio Villegas, Ciudad De México, México, 2 Laboratorio HLA, Instituto Nacional de Enfermedades Respiratorias, Ismael Cosío Villegas, Ciudad de México, México, 3Laboratorio de Investigación Traslacional en Envejecimiento y Fibrosis Pulmonar, Instituto Nacional de Enfermedaedes Respiratorias, Ismael Cosío Villegas, Ciudad de México, México.

Objectives: The association of antisynthetase antibodies (ASAB) with either interstitial lung disease (ILD) and/or inflammatory myopathy (IM) is known as antisynthetase syndrome (ASSD). ILD is the most serious manifestation of ASSD with high morbidity and mortality. We performed this study to estimate levels of serum of IL-18, IL-1β and sCD163, before and after treatment with methotrexate, leflunomide and prednisone in patients with ASSD.

Methods: Patients with ILD all positive to ASAB. All patients were treated at a single center with methotrexate, leflunomide and prednisone. We examined the clinical course, pulmonary function tests, pattern of interstitial lung disease by HRCT, and baseline levels and at follow up of IL-18, IL-1β, TGFβ-1 and sCD163 by ELISA.

Results: 40 patients were included. Most patients were Jo1 positive (45%), other ASAB 55%. Eighty percent of the patients had improvement in pulmonary disease at follow up (improvement in FVC > 10% of predicted and/or improvement in DCLO > 15% of predicted). IL-1β, IL-18 and sCD163 had a decrease in levels at follow up (12 months): baseline IL-1β 46 (31-91) pg/ml Vs follow up IL-1β 21 (15- 30) pg/ml, p < 0.0001; baseline IL-18 158 (74-324) pg/ml Vs. follow up IL-18 77 (41-120) pg/ml, p < 0.0001; baseline sCD163 67182 ± 38548 pg/ml, Vs. follow up sCD163 31020 ± 16142 pg/ml, p < 0.0001. On the contrary, levels of TGFβ-1 increased after at follow up: baseline TGFβ-1 56 (52-61) pg/ml, Vs. follow up 65 (60-69) pg/ml, p < 0.001. Patients with lung disease progression (n:5) had a tendency towards higher levels of sCD163 at baseline (p=0.09) and both at follow up (p=0.07).

Conclusions: These results suggest, that after treatment there is a decrease in the levels of pro inflammatory cytokines (IL-1β and IL-18), with an increase in regulatory TGFβ-1. There was a tendency towards higher levels of sCD163 in patients with progressive lung disease, both at baseline and at follow up, so it may be a biomarker of bad prognosis in patients with ILD positive to ASAB.

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Diana Paola Wilches Reyes1, Mario Javier Moreno Álvarez1, and Rafael Ignacio López Martínez1. 1Hospital Luis Vernaza, Guayaquil, Ecuador.

Objectives: 1.- To determine the relationship between nailfold capillaroscopy patterns (NFC) and modified Medsger severity scale (MSS) in patients with Systemic Sclerosis (SSc). 2.- To identify the relationship between SSc and NFC specific autoantibodies. 3.- To link together the MSS and the years of evolution of the disease. 4.- To determine the relationship between modified Rodnan skin score (mRSS) with NFC.

Methods: Observational, analytical, descriptive, cross-sectional study in adult patients with SSc seen at Luis Vernaza outpatient Clinic from December 2017 to May 2018. The capillary alterations were correlated with MSS. The data was analyzed by MedCalc Statical Software 18.2.1 version.

Results: 49 patients were studied, 38 with limited SSc (77.55%) and 11 with diffuse SSc (22.44%), average age was 49,5 years; 91.8% were women. Patients with late NFC had greater systemic involvement (P=0.0444), Spearman 0.30 rho coefficient. There was no association between the different NFC and auto autoantibodies specific to SSc (p= 0.6045). There was no evidence of correlation between years of evolution of the disease and MSS, Kendall´s Tau 0.66 (95% CI 0.124-0.258; p=0.4918). In evaluating the correlation between MSS score and NFC, this was found to be significant (p=0.0490).

Conclusions: There is a strong relationship between NFC and the severity of visceral damage in patients with SSc. The severity of the disease is not influenced by the years of evolution or by the presence of autoantibodies. Cutaneous involvement seems to accompany the microangiopathic changes evaluated by capillaroscopy. The periodic evaluation of NFC in autoimmune rheumatic diseases and in particular in SSc, is a tool of great diagnostic and prognostic value.

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Patricia Jordan-Gonzalez1, Ricardo Gago-Piñero1, Irma Vázquez-Sanabria1, Noemi Varela-Rosario1, Naydi Perez-Rios1, and Luis Vila1. 1University of Puerto Rico Medical Sciences Campus, San Juan, Puerto Rico.

Objectives: C3 and C4 hypocomplementemia are predictors of lymphoma in patients with primary Sjögren's syndrome (pSS). Also, some studies have shown associations with disease damage. However, the association with clinical manifestations and other comorbidities is not well established. Therefore, we determined the clinical correlates of C3 and C4 hypocomplementemia in a group of patients with pSS.

Methods: A cross-sectional study was performed in Hispanics from Puerto Rico with pSS. All patients were ≥ 21 years of age and met the 2012 American College of Rheumatology Classification Criteria for pSS. Demographic features, health-related behaviors, clinical manifestations, autoantibodies, comorbidities, pharmacologic treatment, disease activity (per EULAR Sjögren's Syndrome Disease Activity Index [ESSDAI]), and disease damage (per Sjögren’s Syndrome Disease Damage Index [SSDDI]) were determined. Serum C3 and C4 levels were measured by immunoturbidimetry at pSS diagnosis. The association of C3 or C4 hypocomplementemia with these factors was studied using bivariate and multivariate logistic regression analyses adjusted for age, sex, and disease duration.

Results: A total of 94 patients were studied, 93.6% were woman. The mean (SD) age and disease duration were 52.4(12.4) and 5.9(4.8) years, respectively. Low C3 and C4 levels were observed in 9.6% and 13.8% patients, respectively. Seven (7.4%) patients had both C3 and C4 hypocomplementemia. Patients with C3 hypocomplementemia were more likely to have leukocytoclastic vasculitis (44.4% vs. 8.2%, p=0.010), interstitial lung disease (33.3% vs. 1.2%, p=0.002), asthma (33.3% vs. 7.1%, p=0.039), and higher SSDDI scores (2.2±2.2 vs. 0.5±0.9, p=0.002) than those with normal C3. Except for asthma, all variables retained significance in the multivariate analysis. Patients with low C4 were more likely to have leukocytoclastic vasculitis (38.5% vs. 7.4%, p=0.007), interstitial lung disease (23.1% vs. 1.2%, p=0.008), and higher ESSDAI (1.5±1.7 vs. 0.6±0.8, p=0.013) and SSDDI (2.00±2.20 vs. 0.48±0.76, p=0.003) scores than those with normal C4. In the multivariate analysis, only interstitial lung disease and disease damage retained significance.

Conclusions: In this population of patients with pSS, C3 and C4 hypocomplementemia were associated with interstitial lung disease and damage accrual. In addition, low C3 levels were associated with leukocytoclastic vasculitis. These data suggest that complements C3 and C4 have clinical and prognostic value in pSS patients.

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Renato Guzman1, Luis Gabriel Piñeros1, Aníbal A. Teherán1, María Camila Mejía Guatibonza1,2, Luis Miguel Pombo1, and Vanessa Cadavid1,2. 1 Fundación Universitaria Juan N. Corpas, Bogotá, Colombia, 2Hospital Universitario Clínica San Rafael, Bogotá, Colombia.

Objectives: Vitamin D (25OHD) has immunomodulatory properties that can play a major role in patients with active lupus or lupus nephritis. Vitamin D’s immunomodulatory function could be influenced by demographic factors, comorbidities (Charlson score), bone supplements, and other features. The aim of our study was to determine the association between the best 25(OH)D cut-off points and specific clinical factors present in patients with active lupus or lupus nephritis.

Methods: A descriptive study using available clinical records of patients diagnosed with systemic lupus erythematosus, being followed at two rheumatology clinics was performed. A tree decision model was used to identify the best cut-off points of 25(OH)D [ng/mL] and clinical features associated with active lupus (SLEDAI-2k >6) or lupus nephritis.

Results: We identified 81 patients, median age 41 years, women 91.3%. Active lupus and lupus nephritis were present in 69.1% and 29.6% of these patients, respectively. Median 25(OH)D was 26.49 ng/mL, without a difference when patients with active and non-active lupus were compared, but lower in lupus nephritis patients 21.50 ng/mL (p=0.015). Lupus nephritis was absent in patients with 25(OH)D cut-off points >38.8 (alone) or ≤38.8 if they were older than 57 years. Lupus was active in patients younger than 44 years with low comorbidity plus cut-off point 25(OH)D >35 and in patients older than 44 years with TSH > 2,11 lU/mL and without Vitamin D suplement (Fig. 1).

Conclusions: Both the activity of the disease and lupus nephritis is influenced by vitamin D levels in interaction with other factors such as age, TSH, comorbidities and the substitution of Vitamin D.

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Manuel F. Ugarte-Gil1,2, Rocío Gamboa-Cárdenas2, Cristina Reátegui-Sokolova2,3, Mariela Medina-Chinchón2, Francisco Zevallos2, Claudia Elera-Fitzcarrald1,2, Victor Pimentel-Quiroz2, Jorge M. Cucho-Venegas2, César Pastor-Asurza2,4, Zoila Rodríguez-Bellido2,4, Graciela S. Alarcón5,6, and Risto Perich-Campos2,4. 1 Universidad Científica Del Sur, Perú, 2 Hospital Guillermo Almenara Irigoyen. EsSalud, Perú, 3 Unidad de Investigación para la Generación y Síntesis de Evidencias en Salud, Universidad San Ignacio de Loyola, Perú, 4 Universidad Nacional Mayor de San Marcos, Perú, 5 University of Alabama at Birmingham, USA, 6Universidad Peruana Cayetano Heredia, Lima, Perú.

Objectives: To determine if achieving LDAS predicts a better HRQoL.

Methods: SLE patients from a single center cohort with at least two visits were included. Visits were performed every six months. HRQoL was measured with the LupusQoL, disease activity with the SLEDAI-2K, damage with the SLICC/ACR damage index (SDI) and comorbidities with the Charlson Comorbidity Index (CCI). LDAS was defined as a SLEDAI-2K≤4, prednisone daily dose ≤7.5mg/d and immunosuppressive drugs on maintenance dose; patients on remission were also included in this group. Generalized estimating equations were performed, using as outcome each one of the eight components of the LupusQoL in the subsequent visit, and the activity state in the previous visit; as possible confounders, we included disease duration, SDI, CCI, antimalarial use on each visit and age at diagnosis, gender, socioeconomic status and the same component of the LupusQoL at the baseline visit.

Results: Two hundred and forty-three patients were included, 225 (92.6%) were female, mean age at diagnosis was 35.44 (SD: 13.13) years. Patients had a mean of 3.94 (1.98) visits for a total of 958 visits. During the follow-up, 590 (61.6%) visits were categorized as LDAS. LDAS predicted a better HRQoL in the components of physical health [B: 4.17 (95%CI: 1.20; 7.14); p=0.006], pain [B: 6.47 (95%CI: 3.18; 9.76); p<0.001], planning [B: 4.97 (95%CI: 1.43; 8.52); p=0.006], burden to others [B: 4.12 (95%CI: 0.24; 8.01); p=0.037], emotional health [B: 4.50 (95%CI: 1.56; 7.44); p=0.003] and fatigue [B: 3.25 (95%CI: 0.04; 6.47); p=0.048].

Conclusions: Being on LDAS predicts a better HRQoL, especially in the components of physical health, pain, planning, burden to others, emotional health and fatigue.

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Manuel F. Ugarte-Gil1,2, Guillermo Pons-Estel3, Luis M. Vila4, Gerald McGwin Jr5, and Graciela S. Alarcón5,6. 1 Universidad Científica Del Sur, Perú, 2 Hospital Guillermo Almenara Irigoyen, EsSalud, Perú, 3 Centro Regional de Enfermedades Autoinmunes y Reumáticas (GO-CREAR), Argentina, 4 The University of Puerto Rico Medical Sciences Campus, San Juan, United States, 5 The University of Alabama at Birmingham, United States, 6Universidad Peruana Cayetano Heredia, Lima, Perú.

Objectives: To determine whether the proportion of time patients achieve either Remission or LDAS is associated with a better QoL.

Methods: SLE patients from a well-established multiethnic, multicenter US cohort were included. Remission and LDAS were defined as follows: Remission, SLAM score=0 and prednisone ≤5 mg/day and no immunosuppressants); LDAS not in remission, SLAM score ≤3, prednisone ≤7.5 mg/day, no immunosuppressants; the proportion of time patients were in these two states (combined) was the independent variable. The end-points were the physical and mental summary measures (PCS and MCS, respectively) and the individual subscales of the Short Form (SF)-36 at the last available visit. Linear regression was used to estimate the association between the proportion of follow-up time in remission and LDAS and the SF-36 measures with and without adjustment for the following baseline variables: age, gender, racial/ethnic group, education, poverty, social support, abnormal illness behaviors, fibromyalgia, disease activity, damage and the baseline scores of the corresponding SF-36 summary measures and subscales.

Results: Five-hundred and forty-two patients with complete data for the dependent, independent and confounding variables were included. These patients were predominantly women, and either of Caucasian, African American or Hispanic ancestry. Overall, the mean scores for the summary measures of the SF-36 were low (38.9 for the PCS and 43.4 for the MCS); for the individual subscales the scores varied between 40.4 for vitality and 65.4 for mental health. In the adjusted MV analysis, the percent of time on either Remission and LDAS was associated with better QoL after adjusting for potential confounders, for PCS the parameter estimate was 9.47 (p<0.0001), for MCS 5.89 (p=0.0027), and for each subscale the estimates ranged between 7.51 (p=0.0495) for mental health and 31.79 (p<0.0001) for role physical.

Conclusions: The percent of time lupus patients stay on Remission or LDAS is associated with a better QoL as measured by the summary measures and subscales of the SF-36.

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Aurelia Luissi1, Marina Scolnik1, and Enrique Roberto Soriano1. 1Hospital Italiano De Buenos Aires, Ciudad Autónoma De Buenos Aires, Argentina.

Objectives: Antiphospholipid Syndrome (APS) is an unusual disease and there are scarce epidemiological data about it. Our objective was to assess the incidence and prevalence rates of APS using data from a tertiary university hospital-based health management organization (HMO) in Latin America.

Methods: Different methods were applied in order to identify possible APS cases: (a) patients with diagnosis of APS, recurrent abortions and/or fetal death in HMO electronic medical records, (b) patients with a Lupus anticoagulant, IgG/IgM anticardiolipin and/or IgG/IgM β2glicoprotein positive test in laboratory database, (c) patients included in the Institutional Registry of Thromboembolic Disease of our hospital. Electronic medical records of all cases retrieved by these ascertainment methods were reviewed and definite APS was diagnosed if the 2006 modified Sapporo Criteria were fulfilled. Global, age-specific, and sex-specific incidence and prevalence rates were calculated for members of the HMO. For the incidence study members with continuous affiliation ≥ 1 year from January 2000 to January 2015 were followed until he/she voluntarily left the HMO, APS was diagnosed, death, or study finalization. Prevalence was calculated on January 1st, 2015.

Results: 53 incident cases of APS were identified during the study period. A total of 349,775 persons contributed a total of 2,073,438 person-years. Incidence rates are reported as cases per 100,000 person-years (py): APS overall incidence rate was 2.6 (CI 1.9-3.2); it was 2.9 (CI 2.0-3.9) and 2.0 (CI 1.1.-3.0) in women and men, respectively. In our population, age-specific incidence and prevalence rates in female patients peaked in the third decades of life and in male they peaked in the sixth decades. On January 1st, 2015, 55 APS prevalent cases were identified from a denominator population of 135,750 HMO members. Prevalence rate was 40.5 per 100,000 persons (CI 29.8-51.2).

Conclusions: Incidence and prevalence rates were similiar to figures from previous reports. Incidence and prevalence rates in women were higher in the young population, and they were associated with obstetric morbidity.

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Elisa Terezinha Hacbarth Freire Elisa1, Danielle Angelieri1, Leandro Parmigiani1, Taciana Paula Sousa Stacchini1, Themis Torres1, Mônica Duarte Costa1, and Paola Fernanda B Toth1. 1Hacbarth Freire Serviços Médicos Ltda, São Paulo, Brazil.

Objectives: Systemic lupus erythematosus (SLE) can affect any organ or system. The activity of the disease is variable, with periods of remission, sometimes long.

AORTITE (Aortitis)– inflammation of the aortic artery wall, with or without rupture of its fibers, has been documented in a few cases, being LUPUS an extremely rare cause of dissecting aneurysm.

We described the case of a patient with grade III aortic dissection, seen in our service and treated clinically, who presented exceptional and no previosly described response to the treatment with rituximab, surviving and having full functional recovery.

Methods: S.M.I.T, Asian-descend, female, 56 years. SLE diagnosed in 1997, immune, hematological, and articular involvement; treated with corticosteroirds (CS), azathioprine with long periods of remission. Stable until August 2017, when she developed a UTI and, after 48 hours, she presented necrosis of the distal phalanx of the 2 ° right finger. Complement consumption, high CRP and ESR, anti DNA +; Angio computerized tomography of the aorta and its branches showed mild ectasia of the pulmonary artery. Increase dose of CS and Azatiotioprina and Cilostazol were given with excellent response. On December 2017 the dose of CS were reduced and Cilostazol discontinued. Later this month patient presented to her primary provider with high intensity lumbar pain. FAN = 1/1280; CRP = 11.6; D dimer = > 10,000; C3 = 55; C4 = 8.4. Angio CT of aorta showed the presence of a thrombus that began in the the aorta’s arch extending inferiorly to the abdominal aorta, with the presence of ulcerated areas.

Results: IV methylprednisolone 1g, followed the next day by Rituximabe 1g IV. Maintained on CS 60mg/day, blood pressure and pain control. Fifteen days after presentation, repeated Angio CTshowed increased ulcerations close to the renal and common Iliac arteries and a positive aneurysmal image in the left iliac. The patient received a second dose of rituximabe 1g. She was discharged from the hospital 72horas after, asymptomatic. On March2018, new Angio CT of aorta showed reabsorption of the intramural hematoma, no ulcerated projections, no areas of stenosis and no atherosclerotic plaques. The patient is well, normotensive, CS have been reduced. After one year, the patient is without evidence of SLE activity, in good health and using low doses of CS. The Angio CT of the aorta performed one year later is normal.

Conclusions: The occurrence of a dissecting aortic aneurysm in patients with active SLE is extremely rare, having been 15 documented cases. The extensive dissection verified, the response obtained with the immunobiological medications used and the patient's survival without accumulation of damage, make this case unique/

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Alberto Christian Ortiz1, Xiomara Cornejo1, Romina Calvo1, Susana Roverano1, and Sergio Paira1. 1Sección Reumatología Hospital JM Cullen, Santa Fe, Argentina.

Objectives: To evaluate SLE activity during pregnancy and postpartum, pregnancy outcomes and to measure pregnancy organic accrual damage in SLE patients.

Methods: We performed a review of the medical records of pregnant patients with a previous diagnosis of SLE (ACR Criteria1997), 16 years or older. We evaluated age of onset, clinical course (since the patient met SLE criteria), time of follow up (since 1st consultation until pregnancy), and clinical manifestations before, during pregnancy and puerperium. Disease activity was assessed using the SLE disease activity index (SLEDAI). Flare was defined as SLEDAI ≥ 3. Damage accrual was measured with the SLICC/ACR Damage Index (SDI) (according to Gladman et al.), before and after pregnancy. Descriptive statistics: X2 test, Fisher’s exact test and Student’s t test were used. Statistical significance was set at p<0.05.

Results: Data of 63 pregnancies from 47 patients were seen between 1997 to 2018. Average age at pregnancy: 27 years (SD:6). Abnormal obstetrical outcome: 25 incidents in 21 pregnancies out of 63 pregnancies reviewed (premature deliveries: 6, threatened abortion: 5, abortions: 4, congenital malformations: 4, low birth weight: 5, fetal demise: 1. Neither a clinical nor a laboratory variable was noted to have the ability to predict any impact on pregnancies’ outcome. In 35% of the pregnancies a flare was reported before pregnancy, 11% during the 1st trimester, 12% during the second trimester, 22% during the 3rd trimester and 14% after delivery. There was no relationship between SLE activity before and during pregnancy (p=0.58). There was an association between SLE activity during pregnancy and low C3 and C4 levels (p=:0.011 and p=0.004). Damage accrual occurred in 40 pregnancies, observing in only 2 of them after pregnancy.

Conclusions: SLE activity during and after pregnancy have no relationship with activity before pregnancy but low complement levels have influence in activity during pregnancy. We did not observe that pregnancy influenced organ damage in patients with SLE. The rate of obstetric complications was lower than described in the literature; we did not find any variables that could impact on them.

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Gisela E. Casillas-Ramos1, Lilia Andrade-Ortega1, and Fedra Irazoque-Palazuelos1. 1Departamento de Reumatología, CMN 20 de Noviembre, ISSSTE, Ciudad de México, México.

Objectives: Lupus Nephritis (LN) is the most significant cause of morbimortality in patients with Systemic Lupus Erythematosus (SLE) even despite recommended treatments. Given the complex pathogenesis of LN, multitarget therapy (MTT) has a logical basis and has been suggested for those cases with bad prognosis or therapeutic failure, with encouraging results.

The objective of this work is to evaluate the response to MTT in LN patients with bad prognosis or failure to treatment during a 2-year period of follow up.

Methods: This work is an analysis of the renal response (using Spanish Guide criteria) and survival in the group of patients from our LN cohort that received MTT and had at least 24 months of follow up. Demographic aspects, characteristics of the disease, histopathological data, and therapeutic history previous to MTT were recorded and correlated with the response to treatment. Statistics analysis was done with SPSS 21.

Results: 20 patients with LN were included in the study, 4 patients (20%) received MTT because of bad prognosis factors since starting therapy, 12 (60%) because of renal relapse and 4 (20%) because of refractory course. After 6/24 months of MTT there was complete response in 25%/75% of the patients, partial response in 50%/15%, and no response in 10%/10%, respectively. Remission at 24 months was associated with baseline albuminuria <1 g/24h and with response at 6 months. No deaths were reported. None of the patients needed dialysis or kidney transplantation and there were only 3 cases with a serious infection in the 2 year follow up period.

Conclusions: In our study, MTT was effective on inducing total or partial renal response on those patients with failure to conventional treatments, without a significant increase in serious adverse events.

Even though our study is small and from the review of medical records its results are encouraging and highlight the need for controlled studies with a larger number of patients in order to corroborate the efficacy and safety of combined treatments. We consider that MTT is promising for refractory LN.

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Karin Daniela Cepeda Armendariz1, Elena Isabel Romero Cevallos1, and Vicenta Teodora Cevallos Carofilis1. 1Laboratorio Interlab S.A., Guayaquil, Ecuador.

Objectives: To determine the prevalence of rare mitotic Anti-Nuclear Antibodies patterns seen in the reports of patients with suspected autoinmune disease or diseases.

Methods: This is an observational study of available data from January to October of 2018 from the Interlab Laboratory of Guayaquil. Data were collected from 6313 serological tests that detected ANA using the method of Indirect Immunofluorescence (IFI), using HEP 2 cells as a substrate. Variables such as sex, age and titer were analyzed. Data were prepared and analyzed by two laboratory professionals. From the 6313 tests, 961 were positive/.

Results: The total number of patients who were ANA positive was 961 (15.2%); of them only 27 (0.43%) were positive for mitotic patterns. Of these 27 patients 81.5% were female and 18.5% male, with ages between 7 and 87 years of age; the mean age for women was 47 years, and 20 years for men. The percentage of cases with dilutions equal or greater than 1: 160 was 95% in the numa-like pattern of 95% and centrosome in 50%.

Numa like: 22 cases

Centrosome: 4 cases

Spindle fibers: 1 case.

Conclusions: This study demonstrates that the prevalence of rare mitotic patterns is 0.43%, which is low. This prevalence is in accordance with other studies. The Numa-like pattern is the most common of the mitotic patterns and the one that included patients from all ages. The centrosome pattern was more common in adults in their middle age. This work could be the start for others aimed at correlating the clinical manifestations with rare ANA patterns, given that the titer and the pattern reflect the activity of the autoimmune process.

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Fernando Afranio Palmeira Oliveira1, Fabiana de Miranda Moura dos Santos1, Maria Veloso Rocha Mameluque1, Thiago Loredo e Silva1, Clara Otoni Neves1, Douglas Mansur Guerra1, Maria Fernanda Botelho Teixeira1, Claudia Lopes Santoro Neiva2, Ana Flavia Madureira de Pádua Dias2, Rosa Weiss Telles1, and Cristina Costa Lanna1. 1 Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, Brazil, 2Santa Casa de Belo Horizonte, Belo Horizonte, Brazil.

Objectives: To investigate the effect of cosmetic camouflage on the health-related quality of life (HRQoL) of systemic lupus erythematosus (SLE) women presenting sequelae of cutaneous manifestations on their face.

Methods: Randomized controlled clinical trial (Universal Trial Number: U1111-1210-2554e) of ambulatory SLE women according to ACR 1997 and/or SLICC 2012 criteria, aged over 18 years presenting sequelae of cutaneous lupus manifestations on their face. Exclusion criteria were: SLEDAI 2k-modified> 4, lack of understanding to questionnaires, psychological and/or psychiatric treatment initiation or modification during the study. A final sample of 43 patients was divided: group I (n=28) patients were trained for cosmetic camouflage use and were instructed to use it daily; and group II (control – n=15) patients did not use any camouflage. SLE Quality of Life (SLEQoL) and Dermatology Quality of Life Index (DLQI) questionnaires were applied to all patients at baseline (T0) and after 12 weeks (T1), higher scores meaning worse HRQoL. The primary end point was a change on HRQoL after camouflage cosmetic use. End points were investigated per-protocol analysis; a p value <0.05 was considered to be significant.

Results: Both groups were similar at baseline regarding age [group I:45.0 (37.3-55.7)years versus group II: 50.0(43.0-55.0)years, p=0.575], disease duration [group I: 17.5 (7.3-26.5) years versus group II: 15.0 (9.0-17.0) years, p=0.452] and SLEDAI-2km [group I: 0 (0-2) versus group II: 2 (0-2), p=0.301]. The DLQI and SLEQoL scores decreased in the cosmetic camouflage group from baseline: DLQI [T0: 8.5 (4.0-16.0) versus T1: 3.0 (1.0-7.5), p=0.008]; SLEQOL [T0: 118.0 (91.0-154.3) versus T1: 95.5 (76.0-135.0), p=0.003]; while there was no variation in the control group. The variation difference of HRQoL scores between groups I and II was statistically significant: ΔDLQI [group I: -3.0 (-10.8-0.0) versus group II: 1.0 (-1.0-6.0), p=0.003] and ΔSLEQoL [group I: -14.5 (-33.0-0.0) versus group II:3.0 (-8.0-10.0), p=0.009]. The SLEQoL score variations were on physical function (p=0.033), humor (p = 0.033) and self-image (p=0.031) domains.

Conclusions: In this group of SLE women with low systemic disease activity and sequelae of cutaneous manifestations, we observed an improvement of HRQoL after daily use of cosmetic camouflage.

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Gelsomina Alle1, John F. Jaramillo Gallego1, Marina Scolnik1, Valeria Scaglioni1, Carlos F. Varela1, Gustavo Greloni1, and Enrique Soriano1. 1Hospital Italiano de Buenos Aires, Ciudad Autónoma de Buenos Aires, Argentina.

Objectives: The aim of this study was to evaluate the predictors of advanced chronic kidney disease (ACKD) in patients with Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis and renal involvement.

Methods: We included all patients with biopsy-proven ANCA glomerulonephritis (GN) between 2002 and 2016. Serum creatinine estimated glomerular filtration rate (eGFR), proteinuria, hematuria, renal histopathology and autoantibodies were recorded at diagnosis, at the end of induction treatment, after one year of treatment and at the end of follow-up, as well as treatments received, requirement of dialysis and renal or extrarenal relapses. Renal biopsies were reviewed and classified according to the classification proposed in 2010 for ANCA GN. Univariate analysis were performed to identify factors associated with long-term ACKD (eGFR <30 ml/min, stages 4 or 5 of KDIGO classification). The diagnostic accuracy for ACKD of each predictor variable were compared using areas under the curve (AUC) of ROC (receiver operating characteristic) curves.

Results: Sixty patients (76.7% women) were included: 17 GPA, 14 MPA, 5 EGPA, 24 RLV; with a mean age of 67.8 years (SD 13.1). Median follow-up time was 4.2 years (IQR 2.2-6.8). At the end of follow-up, 12 patients (20.7%) had an eGFR<30ml/min. In the univariate analysis the serum creatinine at diagnosis (OR 1.24, 95%CI 1.02-1.52, p0.03), end of induction (OR 15.4, 95%CI 2.41-98.28, p0.004), and 12 months (OR 19.25, 95%CI 2.75-134.92, p0.003), and the sclerotic class biopsy (OR 7.17, 95%CI 1.34-38.31, p0.02), were significantly associated with ACKD. The best diagnostic accuracy in ROC curves was shown by serum creatinine at the end of induction (AUC 0.93, 95%CI 0.83-1.00) and at 12 months (AUC 0.94, 95%CI 0.85-1.00).

Conclusions: In this cohort of patients with ANCA vasculitis and renal involvement, serum creatinine at the end of the induction period and after 12 months of treatment were the best predictors of ACKD (eGFR <30ml/min) at the end of follow-up.

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Susana Roverano1, Romina Calvo1, Alberto Ortiz1, Sergio Paira1, Lucas Costa2, and Elena Carrera2. 1 Hospital José María Cullen, Santa Fe, Argentina, 2Unidad Bioestadística Aplicada, Univ. Nacional del Litoral- Fac. de Ciencias Médicas, Santa Fe, Argentina.

Objectives: The incidence of Systemic Lupus Erythematosus (SLE) in males ranges from 4 to 22%. The disease is more frequent in females than in males (9:1). Male SLE carries a worse prognosis.

Arthritis (53%), anti-ds-DNA antibodies (46%), serositis (34%) and malar rash (27%) are the most prevalent findings in males at the onset of SLE.

The aim of this study was to determine gender differences in our SLE patients.

Methods: Material and methods: observational and historically study carried on in a single center.

We included all patients with SLE ACR criteria 1984 and 1997, taking into account the following variables at first consultation: sex, age, time of evolution and of follow up, symptoms and laboratory data (Erythrosedimentation Rate, Hemolytic Anemia, Leukopenia, Lymphopenia, Thrombocytopenia, ANA (HeP-2), anti-ds-DNA antibody, low C3 and C4, anti-Ro, La, Sm, RNP, anticardiolipins and Lupus Anticoagulant). Outcomes of both groups (SLE female and SLE male) were compared.

Results: 267 females and 34 males were identified, with similar age at SLE onset (29 years old). Females had a longer time of follow up (5,87 vs. 3,14 years p=0,0001) and a longer time since the presentation of the first symptoms due to SLE until consultation (40 vs 14 months p=0,001) than males. Alopecia was more frequent in males than in females (47 vs 29,6% p=0,039) but malar rash (32 vs 64% p=0,0001) and anti-Ro Antibodies (17,6 vs 31,5% p=0,02) were less frequent.

There were no diferences between groups in terms of treatment received, SLICC-SDI, SLEDAI at 1st visit, mortality (13,5% females vs. 17.6% males) and outcomes.

Conclusions: Similarly, to the data for the GLADEL cohort, our male patients presented at the same age and the delay to diagnosis was shorter than in females. SLE male patients of both GLADEL and Taiwan cohorts showed renal involvement with low C3 and higher mortality than female patients, unlike our study that showed Alopecia to be more frequent in males but males had similar outcomes than the female patients. There was no statistical difference in mortality between both groups.

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Florencia Vivero1, Federico Campins1, Silvia Babini1, Pablo Malfante1, Diana Lancellotti1, Esteban Gándara1, Javier Sebastiani1, Victoria Basso1, Juan Ignacio Enghelmayer2, Adrián Gaser3, Norma Naval4, Mariana Lagrutta5, Soledad Altube6, Viviana Moyano7, Mónica Sacnun8, Javier Abdala9, Hernan Basilo Vigil10, Lilian Capone11, Carolina Isnardi12, Adriana Robles13, Magdalena Romiti1, Marina Oliver1, and Mariela Bastita1. 1 Hospital Privado De Comunidad, Mar Del Plata, Argentina, 2 Hospital de Clínicas, Buenos Aires, Argentina, 3 Instituto Diagnóstico Médico, Buenos Aires, Argentina, 4 Hospital Ángel Padilla, San Miguel de Tucumán, Argentina, 5 Hospital Centenario, Rosario, Argentina, 6 Hospital Municipal, Chivilcoy, Argentina, 7 Hospital Italiano, Córdoba, Argentina, 8 Hospital Provincial, Rosario, Argentina, 9 Hospital Central, Mendoza, Argentina, 10 Hospital Tornú, Buenos Aires, Argentina, 11 Instituto Vaccarezza, Buenos Aires, Argentina, 12 Instituto de Rehabilitación Psicofísica, Buenos Aires, Argentina, 13Hospital San Bernardo, Salta, Argentina.

Objectives: The aim of this study was to describe the demographic, serological, imaging, and lung functional features along with the treatments for patients with diffuse interstitial lung disease (ILD) associated with systemic lupus erythematosus (SLE).

Methods: Descriptive, multicenter study. Patients from the EPIMAR cohort were included. Participants were recruited at 25 centers from Argentina that provided multidisciplinary care for ILD patients. Patients evaluated between January 2015 and April 2018 and diagnosed with ILD defined by the presence of suggestive pulmonary images on high-resolution computed tomography (HRCT) (irregular inter or intralobular septal thickening, traction bronchiectasis, cystic and/or ground glass images) associated with SLE according to 1997 ACR criteria were included. Serological data and studies of respiratory function (spirometry and DLCO) were recorded. All the images were blindly evaluated by a diagnostic imaging specialist with practice in chest diseases who defined the tomographic pattern. Data on treatment schemes were recorded.

Results: Out of the 381 patients recruited in the EPIMAR cohort, 29 (7.6%) presented SLE; and 90% (26/29) were women. Age ranged from 29 to 80 years with an average of 53 years (SD 12). Autoantibody frequency was ANAs: 93% (27/29), anti-Ro/SSA: 58% (15/26), and anti-RNP: 35% (9/26). The tomographic patterns found were: non-specific interstitial pneumonia (NSIP): 28% (8/29), usual interstitial pneumonia (UIP): 24% (7/29), NSIP/organized pneumonia (OP) overlap: 10% (3/29). At the time of diagnosis, the percentage of forced vital capacity (% FVC) was 66% (SD 17.58) and DLCO was 56% (SD 19.83). Treatment data were obtained for 83% of patients (24/29). All patients received corticosteroids. The immunosuppressants used were: 38% cyclophosphamide (9/24), followed by 25% azathioprine (6/24), and 21% mycophenolate (5/24). Outcome data were obtained (% FVC at 6-12 months) in 50% of cases, with stability or improvement of lung function in 78% (11/14) of the treated patients.

Conclusions: To the best of our knowledge, this study recruited the largest number of lupus patients with ILD. SLE-associated ILD is less frequent than in other connective tissue diseases. This SLE association was primarily found in middle-aged, non-smoker women with a significant prevalence of anti-Ro/SSA and a lower frequency of ANAs, in line with the data reported in other series. Longitudinal studies should be conducted to validate these results.

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José Raúl Lambertino-Montaño2, Carlos Jaime Velásquez-Franco1,4, Jorge Hernán Gutiérrez-Marín3,4, María Nazareth Campo-Campo3,4, José Enrique Sanín-Blair3,4, Raúl Alejandro García3,4, Simón Hoyos-Patiño4, Sara Manuela Ocampo-Ramírez4, Lady Johanna Hernández Zapata1,5, Libia María Rodríguez-Padilla4, and Miguel Antonio Mesa Navas1,4. 1 Clínica Universitaria Bolivariana, Medellín, Colombia, 2 Clínica SOMER, Rionegro, Colombia, 3 Clínica Universitaria Bolivariana, Colombia, 4 School of Health Sciences. Universidad Pontificia Bolivariana, Medellin, Colombia, 5Universidad de Antioquia, Medellin, Colombia.

Objectives: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that predominantly affects women of reproductive age, being the coexistence of SLE and pregnancy a common situation. This condition, if it is not in remission prior to pregnancy, can become active in a serious way during pregnancy and the immediate postnatal period, increasing the risk of maternal and fetal complications. The objective of this survey was to evaluate the epidemiological, clinical and therapeutic features of pregnant women with SLE, as well as their obstetric outcomes.

Methods: A cohort of pregnant women with SLE from the Maternal and Fetal Medicine and Rheumatology Units in two high-complexity hospitals from Medellin, Colombia, between 2010 and 2015, was constituted and evaluated. Qualitative variables were expressed in absolute and relative frequencies, while quantitative ones with mean and standard deviation.

Results: Forty patients and 43 pregnancies were included, with an average age at diagnosis of SLE of 24 ± 7 years. Joint involvement was the most frequent clinical manifestation (n=32; 80%). Only 8/43 (18.6%) of lupus pregnancies were active at the beginning of gestation. The most frequent obstetric complication was preeclampsia (11/43; 25.6%). The most common fetal complication was preterm birth (8/43; 18.6%); in this scenario, anticardiolipin and anti-DNA antibodies were positive in 10/43 (23.3%) and 15/38 (39.5%) pregnancies, respectively. In 13/43 (31.7%) pregnancies, a relapse of SLE during the third trimester occurred; six of them (46.1%) were classified as severe, mainly nephritis. On admission to antenatal control, 29/43 (65%) women were under treatment with prednisone, 21 with chloroquine (65.6%), and 11 (34.4%) with hydroxychloroquine. There was an increase in the average dose of prednisone during pregnancy of 20%. During the postnatal period, two patients had moderate lupus relapse.

Conclusions: Lupus nephritis and antiphospholipid antibodies were present in a significant proportion of the patients with obstetric complications. Even though a low percentage of patients presented lupus activity prior to pregnancy, maternal and fetal complications were frequent. Although the use of hydroxychloroquine during pregnancy is reported in the literature, the majority of women in this cohort received chloroquine, without a significant increase in lupus activity or the occurrence of obstetric complications.

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Ricardo Bedón1. 1 Hospital General Docente De Calderón, Quito, Ecuador, 2Universidad Central del Ecuador, Quito, Ecuador.

Objectives: The monogenic autoinflammatory diseases (AIFD) are characterized by recurrent or persistent episodes of fever, joint pain and other varied symptoms. They are caused by mutations in genes related to some inflammatory pathways. The presence of recurrent or continuous fever, as a guiding sign, in patients in whom an infectious and tumoral autoimmune cause could be ruled out and where the genetic study of the main ones AIFD is negative, confers an undifferentiated autoinflammatory genotype.

OBJECTIVES: To describe the clinical and biological characteristics of a cohort of patients with undifferentiated autoinflammatory diseases.

Methods: Analysis of recorded data (2014- 2016) of adult patients with undifferentiated AIFD from a unit of autoinflammatory diseases of the autoimmune diseases service of the Hospital Clínico de Barcelona. In order to identify and characterize the different clinical phenotypes, clinical and analytical data were obtained, at the time of diagnosis and during follow-up, as well as information on the treatments used and their efficacy.

Results: Of 107 patients suspected of having AIFD, 30 (28%) of them were diagnosed as Undifferentiated AIFD. Among them, the average age at the begining of the clinical picture was 27±14 years, with a predominance of women (63 %). Based on the prevailing symptoms, four phenotypes or complex systemic clinical syndromes were determined ( 47 % ), syndrome of periodic fever, aphthous stomatitis, pharyngitis and atypical adenitis (PFAPA atypical) ( 23% ), recurrent pericarditis ( 20% ) and Familiar Mediterranean Fever - like ( FMF- like ) ( 10% ).The drugs to which patients finally had an adequate response were colchicine ( 33% ), prednisone on demand (20% ) and Anakinra (anti- interleukin -1) ( 27% ). Patients with complex systemic pictures more frequently required (50%) Anakinra. In 20% of the patients a self-limiting course, was observed without requiring a specific treatment.

Conclusions: The undifferentiated AIFD can be grouped into characteristic phenotypes according to the predominant symptoms. Within the treatments used, Anakinra was the most common in those cases considered more complex.

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Guillermo Valencia Pacheco1, Yumi E. Nakazawa Ueji1, Julián Ramirez Bello2, Rosa E. Barbosa Cobos3, Eduardo D. Jiménez Beccerra1, Gerardo J. Perez Mendoza1, and Nubia A. Rivero Cardenas1. 1 Laboratorio de Hematología. Centro de Investigaciones Regionales Dr. Hideyo Noguchi, de la Universidad Autónoma, Yucatán, México, Mérida, 2 Research Unit on Endocrine and Metabolic Diseases Hospital Juárez de México, Ciudad de Mexico, Mexico, 3Hospital Juárez de México, Ciudad de México, México.

Objectives: The mestizos of Yucatan are the only group whose Amerindian contribution is mainly Mayan, and they represent an ethnic group geographically distant from other Mexican Amerindian groups. Due to heterogeneity among mestizo subpopulations from geographically distant regions of Mexico, the study of genetic factors such as Copy Number Variation (CNV) or the Single Nucleotide Polymorphysm (SNP) in the TLR7 gene associated with the pathogenesis of SLE is important.

Objective: to analyze the CNV and SNP rs179008 (A> T) of the TLR7 gene and its association with with the development of SLE in mestizo patients from Yucatán and Mexico City.

Methods: The study was conduced including 151 female patients with SLE and 121 healthy female controls from Mexico City, and 100 female patients with SLE and 102 helathy females controls from Yucatan, who voluntarily agreed to participate in the study. Both CNVs and genotyping of SNP rs179008 (A>T) was determined by real-time PCR with TaqMan probes. The estimation of CNV was made by calculating 2-ΔΔCt. The allelic and genotypic frequencies were analyzed using the STATA 10 program. The association of CNV and SNP rs179008 (A>T) with the disease was analyzed by determining the Odds Ratio (OR) with a confidence interval of 95%, and the results were contrasted by the Chisquare test.

Results: We observed that 14% of the patients from Yucatán showed significantly more than 2 copies with respect to 4% of those from Mexico City. The association analysis shows that the Yucatecan women presents a higher risk of developing the disease (OR = 34.36, p = 0.0003) than those from Mexico City (OR = 10.85, p = 0.07). Regarding the SNP rs179008 (A> T), the wild allele A was found more frequent in patients and controls of Yucatán (74% and 76%) and Mexico City (51%) and 61%), while the T risk allele was less frequent. However, the T allele shows greater association with the disease in the patients from Mexico City (OR = 1.53, p = 0.01). The mutated A / T genotype presented a greater frequency and association with the disease in patients from Mexico City (OR = 9.75, p <0.0001).

Conclusions: Genetic studies of association and expression, based on the search of CNV and presence of SNPs of a gene, constitute a valuable tool to better understand the etiopathogenesis of the disease and identify genetic markers.

The results support the role of the TLR7 gene in the pathogenesis of SLE in Mexican mestizo populations and suggest that extra copies of the gene may be a risk factor for developing the disease. The presence of the mutated A / T genotype associated with the disease, mainly in patients from Mexico City, supports the genetic differences between and within Mexican mestizo populations.

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Manuel Ugarte-Gil1,2, Guillermo Pons-Estel3, Russell Griffin4, Guillermina Harvey5, Daniel Wojdyla5, Luis M. Vilá6, Loreto Massardo7, Eloisa Bonfá8, Mario Cardiel9, Verónica Saurit10, Enrique Soriano11, Bernardo Pons-Estel3, and Graciela S. Alarcón12,13. 1 Universidad Científica Del Sur, Santiago De Surco, Perú, 2 Hospital Guillermo Almenara Irigoyen. EsSalud, La Victoria, Perú, 3 Grupo Oroño - Centro Regional de Enfermedades Autoinmunes y Reumáticas (GO-CREAR), Rosario, Argentina, 4 Department of Epidemiology, University of Alabama at Birmingham, Birmingham, US, 5 Facultad de Ciencias Económicas y Estadística, Universidad Nacional de Rosario, Rosario, Argentina, 6 Division of Rheumatology. University of Puerto Rico, San Juan, US, 7 Centro de Biología Celular y Biomedicina, Facultad de Medicina y Ciencia. Universidad San Sebastián, Santiago, Chile, 8 Faculdade de Medicina, Hospital das Clinicas HCFMUSP, Universidade de São Paulo, Sao Paulo, Brazil, 9 Centro de Investigación Clínica de Morelia, Morelia, Mexico, 10 Servicio de Reumatología, Hospital Privado Universitario de Córdoba, Cordoba, Argentina, 11 Sección de Reumatología, Servicio de Clínica Médica, Instituto Universitario, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina, 12 School of Medicine. The University of Alabama at Birmingham, Birmingham, US, 13Facultad de Medicina. Universidad Peruana Cayetano Heredia, Rímac, Lima, Perú.

Objectives: To determine the sensitivity of the 2018 EULAR/ACR criteria using two well-defined lupus cohorts.

Methods: Patients from two multiethnic and multicenter lupus cohorts, one from the US and the other from Latin-America were included. SLE patients were classified using the 1982/1997 ACR criteria in the US cohort and as diagnosed by expert physicians in lupus for the Latin-American cohort although more than 95% of these patients also achieved the 1982/1997 ACR criteria. For these analyses the 1982/1997 ACR criteria were used as the gold standard. Demographics and disease activity at baseline (measured with the SLAM for the US cohort, and with the SLEDAI for the Latin-American cohort) were compared in order to determine differences between those patients who achieved the EULAR/ACR criteria before, at the same time or after than 1982/1997 ACR criteria.

Results: Five-hundred and fifty-eight patients out of 640 from the US cohort and 956 out of 1047 from the Latin-American cohort achieved the EULAR/ACR criteria. The sensitivity of the EULAR/ACR criteria in the US cohort was 87.2% and in the Latin-American cohort was 91.3%; in the US cohort, 41 (7.3%) achieved the EULAR/ACR criteria earlier, 344 (61.6%) at the same time and 173 (31.0%) later than the ACR criteria; for the Latin-American cohort these numbers and percentages were 71 (7.4%), 556 (58.2%) and 329 (34.4%), respectively. Patients who achieved the EULAR/ACR criteria earlier were less likely to be Caucasian (12.2%) in the US cohort and 30.0% in the Latin-American cohort compared to those who achieved both criteria at the same time (21.2% and 52.0%) or the EULAR/ACR criteria later (37.6% and 47.0%); disease activity did not differ between the groups.

Conclusions: The sensitivity of the 2018 EULAR/ACR criteria vs. the 1982/1997 ACR criteria (gold standard) was high (87.2% for the USA and 91.3% for the Latin-American cohort). While the large majority of patients were classified at the same time (58.2% to 61.6%) with both criteria, about one third were classified later and a small proportion (about 7%) were classified earlier with the 2018 EULAR/ACR criteria. In this last group, the EULAR/ACR criteria seem to perform better in non-Caucasian populations. Further examination of the 2018 EULAR/ACR criteria is warranted.

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Susana Roverano1, Romina Calvo1, Alberto Ortiz1, Sergio Paira1, Lucas Costa2, and Elena Carrera2. 1 Hospital José María Cullen, Santa Fe, Argentina, 2Unidad Bioestadística Aplicada-UNL-FCM, Santa Fe, Argentina.

Objectives: Survival in Systemic Lupus Erythematosus (SLE) has improved in the last decades, hence, organ damage tends to accumulate over time.

We undertook this study in order to determine organ damage accumulation in patients who had been followed (at least some of them) for twenty - five years.

Methods: All SLE patients were followed at the Rheumatology Section of Cullen Hospital, (established in 1987). We took into consideration demographic data, time of follow up, treatment received, and SLICC-SDI calculated at 1st, 2nd, 5th, 10th, 15th, 20th and 25th year of follow up.

Results: 301 patients were evaluated; mean time of follow up: 5.52 years SD: 4.84-6.20) and mean age at diagnosis: 32.43 ± 12.6 years. Almost 50% of the patients had an SDI=0 at the 5th year of follow up. Organ damage increased over time: 1.58 at 1st year to 3.70 at 25th year. Renal, Musculoskeletal and Neuropsychiatric domains were the most involved in this follow up, while Gastrointestinal, Gonadal, Malignancies and Peripheral Vascular were less preponderant. Proteinuria, Arthritis and Seizures were the most common subtypes observed.

Conclusions: Gladman et al and Urowitz et al described early damage in Cardiovascular, Gastrointestinal and Skin domains. In our study Renal and Musculoskeletal System damage were the more commonly found. Treatments are more effective in SLE patients. Damage accrual may result from either treatment received or the disease itself.

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Michelle Fuseau Herrera1, David Garrido Salazar2, Líder Escudero Abad2, and Diego Noboa Torres2,3. 1 Universidad de la República, Montevideo, Uruguay, 2 Hospital de Especialidades de las Fuerzas Armadas N°1, Quito, Ecuador, 3Universidad Central del Ecuador, Quito, Ecuador.

Objectives: To determine the prevalence of hospital discharges due to systemic connective tissue disorders in Ecuador, as well as the specific characteristics of patients and their geographical distribution.

Methods: This is a transversal descriptive study conducted in Quito, Ecuador. Based on the analysis of discharge hospitalization diagnosis, coded according to the International Classification of Diseases (ICD) as Systemic Connective Tissue Disorders (SCTD), which includes polyarteritis nodosa and related conditions (M30), other necrotizing vasculopathies (M31), systemic lupus erythematosus (M32), dermatopolymyositis (M33), systemic sclerosis (M34), other systemic involvement of connective tissue (M35), systemic disorders of connective tissue in diseases classified elsewhere (M36); from a secondary source published by the Instituto Nacional de Estadisticas y Censos (INEC, National Institute of Statistics and Census) of Ecuador entitled “Registro Estadístico de Camas y Egresos Hospitalarios, 2017”, which presents information of all the Ecuadorian public and private health establishments with hospitalization, collected during 2017. Data were analyzed by sex, age groups, and province of residence.

Prevalence was calculated by 100000 inhabitants using the tabulated data through Excel, using the latest version of SPSS software package.

Results: Of 1482 patients found, 1173 (79.15%) were female, being the prevalent group in all the diagnostics (female-to-male ratio 4:1) excepting in Polyarteritis nodosa and related conditions, where male children from 1 to 4 years were the prevalent group.

A higher prevalence during the reproductive years was observed; peaking between 23 and 34 years with 344 patients (23.48%), followed by individuals between 35 and 44 years (17.54%). The most frequent diagnosis was systemic lupus erythematosus (SLE) with 1125 cases (76%), from them, 973 (86.49%) were female (female-to-male ratio 6:1).

We found higher prevalence rates of SCTD by 100000 inhabitants in Pichincha 19.67 and Imbabura 11.36, contrasting to the rest of the country which prevalence rates varies from 0.80 in Napo to 9.30 in Carchi. The difference between the observed means was -9.99 (95% CI -14.2351 to -5.7449; p-value < 0.0001), reflecting a significantly higher prevalence of SCTD in Pichincha and Imbabura.

Conclusions: SCTD are more frequent in women, during the reproductive years. The only predominance of male observed was in children with ICD code M30, surely corresponding to the subcategory Mucocutaneous lymph node syndrome (Kawasaki).

Despite not having the diagnoses distribution by province, SLE was the predominant diagnosis.

The significantly higher prevalence rates of SCTD detected in Pichincha and Imbabura requires the strengthening of medical personnel and hospitals for identifying and managing these pathologies, especially in these provinces.

Demographic data and possible predisposing factors have to be investigated and correlated in further studies.

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Oscar Andrés Caicedo Correa1, Silvia Papasidero1, Silvana Karina Pérez1, Juan Bande1, Alejandra Medina1, Julia Sosa1, José Angel Caracciolo1, Rafael Chaparro Del Moral1, Anastasia Secco2, Diego Puente Trigo2, Marina Gauna2, Antonio Catalan Pellet2, Oscar Rillo3, Alejandro Nitsche4, Catherine Crow4, Andrea Gómez5, Federico Zazzetti5, Sofía Vélez5, Juan Carlos Barreira5, Nadia Raquel Benzaquén6, Laura Encinas6, Francisco Caeiro6, Eduardo Albiero7, Natalia Tamborenea8, Gabriela Salvatierra9, and Laura Raiti10. 1 Hospital General de Agudos Dr. "Enrique Tornu", Buenos Aires, Argentina, 2 Hospital General de Agudos "Bernardino Rivadavia", C.A.B.A., Argentina, 3 Hospital General de Agudos Dr. “ Ignacio Pirovano”, C.A.B.A., Argentina, 4 Hospital Alemán, C.A.B.A., Argentina, 5 Hospital Británico, C.A.B.A., Argentina, 6 Hospital Privado De Córdoba, Córdoba, Argentina, 7 Hospital Córdoba, Córdoba, Argentina, 8 Organización Médica de Investigación, C.A.B.A., Argentina, 9 Instituto Provincial de Rehabilitación Integral, Santiago del Estero, Argentina, 10Clínica Bessone, San Miguel, Argentina.

Objectives: Primary Sjögren's Syndrome (pSS) is a chronic autoimmune disease characterized by progressive mononuclear infiltration of the exocrine glands, presenting oral and ocular dryness as main clinical manifestations. A variety of organs can be affected, being the joints frequently involved. The aim of this study was to describe the frequency of arthritis and arthralgia in patients with pSS; to evaluate the joint involvement, and to analyze its association with other clinical and serological manifestations.

Methods: Multicentre, observational and analytical study. Patients with pSS (ACR/EULAR criteria) included in the pSS study group database from the Argentine Society of Rheumatology. Demographic data, clinical manifestations, laboratory findings and complementary studies were recorded. In order to compare patients with joint involvement (arthralgia and/or arthritis) vs those without it, individuals who subsequently developed rheumatoid arthritis (RA) or other connective tissue disorder were excluded. A subgroup of patients with joint involvement was analyzed to characterize the joint involvement.

Results: Two hundred and fifty-nine patients were analyzed: 91% women, mean age 58 years (SD 3.5). Seventy percent presented arthralgia or arthritis. Joint involvement was analyzed in 86 patients. A greater frequency of metacarpophalangeal and carpus involvement was observed in the arthritis group (56% vs 50%). In the group with arthralgia, the most affected joints were knees (40%) and proximal interphalangeal (40%). Polyarticular and intermittent involvement was the predominant pattern. Erosive lesions were reported in 3 patients, only 1 met criteria for classification for RA. When compared with the group without joint involvement, some statistically significant differences were observed: greater proportion of parotid swelling (40% vs 20%, p = 0.004) and positive RF (66% vs 44%, p = 0.002). In the logistic regression analysis, the variables associated with joint manifestations were: RF + (OR 2.5, 95% IC: 1.3-4.7) and parotid swelling (OR 2.4, 95% IC: 1.1-5.0).

Conclusions: We found that 70% of patients with pSS had joint involvement and this was associated with the presence of parotid swelling and positive RF. In addition, we highlight the finding of erosive lesions (as they are rare in pSS) in 3 patients.

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Daniel Rivas-Vargas1, Francisco Bustamante1, Soham Al Snih2, and Martin Alberto Rodríguez1. 1 Hospital Universitario De Caracas, Caracas, Venezuela, 2División of Rehabilitation Sciences, University of Texas Medical Branch, Galveston, Estados Unidos.

Objectives: To determine the association between body mass index (BMI) and interstitial lung disease (ILD) in patients with scleroderma.

Methods: A cross-sectional, correlational, non-probabilistic sample, of consecutive patients with scleroderma seen in the Rheumatology Division at the Hospital Universitario de Caracas during the period of April to September 2018. Patients underwent clinical and laboratory assessments, anthropometric measurements, pulmonary function tests, high-resolution computed axial tomography scan of the chest and full-body densitometry.

Results: Of 48 patients, 95.8% were female, 64.6% had limited cutaneous scleroderma. Underweight [Body Mass Index (BMI) < 18.5 Kg/m2] was a risk factor associated with ILD (OR 4.60, 95% CI 1.12-18.86). BMI was related in a fashion directly proportional to forced vital capacity (FVC) (CC 0.35, p=0.0152). Predictors of ILD included diffuse scleroderma (OR 6.91, 95% CI 1.78-26.85), modified Rodnan Skin Score (mRSS) (OR 1.25, 95% CI 1.04-1.30) and a speckled pattern of antinuclear antibody (ANA) (OR 5.62, 95% CI 1.33-23.62). Multivariate analysis showed that mRSS (OR 1.34, 95% CI 1.04-1.72) and the presence of a speckled ANA (OR 25.99, 95% CI 1.02-662.02) were independent predictors of ILD. The percentage of BMI lost in a period of time of 2008-2018 was 14.3% (p=0.0021), which was higher than in patients with rheumatoid arthritis (p=0.0000), systemic lupus erythematosus (p=0.0025) and healthy subjects(p=0.0331)

Conclusions: Underweight was a risk factor associated with ILD in scleroderma patients. The mRSS could be used as a clinical predictor tool for ILD in scleroderma.

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Janeth Villegas Guzmán1, and Bety Yañez Alvarez1. 1Hospital Nacional Dos de Mayo, Lima, Perú.

Objectives: To determine the frecuency and type of ophthalmologic manifestations in systemic sclerosis (SSc) and establich their association with the extent and severity of cutaneous fibrosis.

Methods: Descriptive – cross-sectional study of 34 patients (January-December 2017). It included patients with Limited cutaneous systemic sclerosis (lcSSc) and Diffuse cutaneous systemic sclerosis (dcSSc). The severity of cutaneous fibrosis was measured using the modified Rodnan skin score (mRSS). Eye examination included: palpebral skin, ocular surface by Schirmer test, time of rupture of teardrop (BUT), the severity of the symptom of dry eye was evaluated through OSDI (Ocular disease surface index) and SANDE (symptom questionnaires assessment in dry eye). The posterior segment eye and retinography fund was evaluated. Applied logistic regression (OR) and Fisher test with p< 0.05 were applied.

Results: 21 patients (61.7%) had dcSSc and 13 patients (38.2%) lcSSc. Women 31 (91.2%). Average time of disease 67.4 months. The mRSS was mild in 14 (41.2%) and moderate - severe in 20 (58.8%). Findings: palpebral fibrosis 30 (88.2%), keratoconjuntivitis sicca (KCS) 26 (76.5%), blepharophimosis 20 (58.8%) and enophthalmos 7 (20.6%), alteration of the retinal pigment epithelium 3 (8.8%). Associated with the extension, dsSSc had significant statistical association with blepharophimosis (p=0.00). KCS mild in both groups with Schirmer test and BUT positive, but no significant association. In terms of the severity of cutaneous fibrosis with mRSS, we found a significant statistical association between enophthalmos (p=0.01) and blepharophimosis (p=0.00) with a moderate- severe score.

Conclusions: The most common alterations of the anterior segment were: fibrosis of the skin of the eyelids and its complications as enophthalmos and blepharophimosis together with KCS. dsSSc patients in moderate to severe extent have high risk of having complications from the palpebral fibrosis: enophthalmos and blepharophimosis. KCS appears to be independent of the severity or the extension.

Eye evaluation and effective treatment of the SSc is important for a better visual prognosis.

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Claudia Maldonado1, Nelson Zambrano1, Mario Moreno1, Enrique Loayza1, and Juan Carlos Garcés1. 1Hospital Luis Vernaza, Guayaquil, Ecuador.

Objectives: Lupus erythematosus panniculitis (LEP) is a type of cutaneous lupus erythematosus that occurs up to 1-3% of patients. Most frequent localizations are upper extremities, trunk and face. Subcutaneous panniculitis T-cell lymphoma (SPTCL) is a rare type of lymphoma represented in less than 1% of skin cell lymphomas, it is common in women and can occur at any age. There are some cases of SPTCL and LEP that demonstrate clinical and histopathologic overlap raising the possibility that they represent opposite ends of a disease spectrum.

Methods: CASE REPORT: Female, 39 years old, with history of choriocarcinoma 14 years before treated with EMACO (etoposide, methotrexate, actinomycin D, cyclophosphamide, vincristine), who presented skin plaques of 12 years in duration, infiltrated with painful nodules, localized in the arms and trunk, accompanied by recent prolonged fever, malaise, asthenia and weight loss. Her laboratory results were negative for ANA, DNA, ENA and low complement. A deep skin biopsy of her left arm revealed a pattern similar to a lobular panniculitis with atypical lymphocytes rimming the adipocytes. CD20, CD3, CD4 markers were negative, but immunohistochemistry for CD8, Ki 67 hotspots were positive which confirmed the diagnosis of SPTCL. The patient was treated with CHOP chemotherapy. Afterward, ultrasonography and histopathologic control only showed a residual panniculitis with dystrophic calcification.

Results: DISCUSSION: SPTCL is a type of cutaneous lymphoma characterized by the subcutaneous infiltration of cytotoxic T cells which have a α/β T-cell phenotype. It affects predominantly young adults. Histologically, the subcutaneous infiltrates simulate a lobular panniculitis with the presence of fat rimming by pleomorphic T cells which is a useful diagnostic feature. Due to both clinical and histological similarity, some authors believe that this is an advanced stage of the same disease in which LEP is transformed into SPTCL. The main difference in the histological diagnosis is that by inmunohistochemistry staining SPTCL is CD8 positive, but LEP is positive for CD123.

Conclusions: This is an unusual case of subcutaneos panniculitis – like t cell lymphoma mimicking lupus erythematosus panniculitis. Given that SPTCL and LEP can show significant overlap, it is necessary to perform complementary studies to make an adequate differentiation.

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Vanessa Castro Coello1, Demelza Yucra Bobarin1, Gabriela Garate Correa1, Adriana Hamaui1, and Diana Dubinsky1. 1Sanatorio Güemes, CABA, Argentina.

Objectives: In SLE there is five times higher risk of cardiovascular events (CV) compared to the general population. In Argentina, the prevalence of metabolic syndrome (MetS) is 28%. The SLICC cohort of early SLE found 36.5%. The neutrophil to lymphocyte ratio (NLR) is a tool in the assessment of CV risk and systemic endothelial dysfunction. The NLR values reported in healthy is 1.65 (±SD 1.47). Studies in SLE show that NLR is a marker of activity and nephritis. We aimed to determine the association of MetS with and without renal involvement, the relationship between NLR with MetS and disease activity.

Methods: Descriptive, cross-sectional study. Patients with SLE (SLICC 2012) of <5 years of disease duration and > 18 years followed at the Güemes Hospital were included, between 06/2013 to 07/2018. Acute cardiovascular events, infections, pregnancy, diabetes and chronic kidney disease were excluded. Traditional risk factors associated with the disease were determined: antiphospholipid (aPL), GC, SLEDAI. MetS was defined according to NCEP ATP III criteria. Laboratory: neutrophil and lymphocyte count, glucose, CT, TG, HDL, LDL, urea, creatinine, proteinuria/24hs, Anti DNA, C3, C4, aPL: LA, B2GPI IgG and Anti aCL antibody. Statistical Analysis: it is presented as means and percentages. T Test or Mann Whitney to compare independent variables according to their distribution. For inferential statistics: Student's t-test and Chi-square test (χ2) for qualitative variables. Statistically significant: p <0.05. Epi Info was used.

Results: A total of 42 patients were reviewed, 12 were excluded (incomplete data). Of 30 patients: 23 women (77%), mean age 39.3 (± SD 14.3), disease duration: 35 months (± SD 18.9), tobacco exposure: 11 (37%). Nephritis in 17 patients (51%). MetS: 13 (43.3%). The correlation of proteinuria of the group with Mets: 1.2 g/d (± SD 3.19) vs the group without MetS 0.35 g/d (± SD 0.54) was statistically significant p = 0.03; likewise, the CRP was higher in the group with MetS vs whitout MetS (p = 0.02). The NLR in MetS was 3.42 (± SD 1.32), in nephritis 3.35 (± SD 1.62) and in patients with SLEDAI ≥ 4 was 4.43 (± SD 1.42).

Conclusions: In our series, a greater frequency of MetS was found than in the literature. No relationship was found between MetS and renal involvement; however, patients with MetS had higher proteinuria and elevated CRP. No relationship was found between the NLR with MetS and nephritis. The NLR was higher in patients with SLEDAI ≥ 4. The high prevalence of MetS, proteinuria, elevated CRP and NLR in our population suggest persistent inflammatory activity and probable CV morbidity, so it is important to detect it from the onset of the disease.

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Diana Gabriela Gárate Correa1, Demelza Yucra1, Ruth Balcazar1, Adriana Hamaui1, and Diana Dubinsky1. 1Sanatorio Guemes, Argentina

Objectives: Rheumatoid factor (RF) in SLE is found in around 25% of patients. Authors associate its presence with cutaneous involvement, Sicca, anti Ro (+) and a protective role in glomerulonephritis (GMN), suggested by the finding of less frequent and less severe nephritis in lupus patients with RF (+) as in patients with RA.

The proposed protective mechanisms of RF include preventing the complement from joining immune-complexes (IC) and avoiding the IC deposition in the glomerulus (1,2,3).

We aimed to determine the clinical and immunological profile of SLE patients with and without RF and to determine the association between RF and GMN.

Methods: Descriptive medical records review study of SLE (SLICC 2012) patients selected from the Rheumatology department of Güemes Hospital, from January 2015-August 2018.

We collected data on demographics, cutaneous-articular, renal, hematological and CNS involvement. Immunoserology: RF IgM by immunoturbidimetric (+) > 30 UI/ml, anti Ro, anti La, anti Sm, anti RNP (ELISA), ANA (Hep 2), Anti DNA (Crithidia lucilliae), C3, C4, aPL at least one of the following: Lupus anticoagulant, anti β2GP1 IgG/IgM, anti aCL IgG/IgM.

Results: We reviewed 147 clinical histories, 107 with RF ordered. Female 93/107 (86.9%), mean age 41.6 years (SD±13.8). Disease duration 98 months (SD±80.6). Tobacco exposure 10/107 (9.35%). RF (+) 22/107 (20.5%), RF median titer 228.8 IU/ml (31-2825 IU/ml).

RF level in patients with GMN was 142 IU/ml (39-191), without GMN 258 IU/ml (31-2825) p= 0.46.

RF level in patients with anti Ro (+) was 248 IU/ml (31-2825), Ro (-) 52.5 IU/ml (31-74) p= 0.37.

RF (-) subgroup showed statistically significant association with female sex, discoid lesions and aPL presence. RF (+) was associated with Ab Ro, La and patients with RA.

No significant association with rash, photosensivity, oral ulcer, joint pain, arthritis, serositis, neurologic and hematological involvement was found.

In those with GMN, we found a significant association with Ab Ro (+) in the absence of RF.

Conclusions: Similar RF frequency was found in our lupus patients as in the published literature. There was no association with GMN, but the mean RF titer was higher in patients without it.

The detection of Ab clusters associated with RF may be useful in suggesting the absence of renal involvement.

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Fiorella Lizeth Vilca Salguero1, Arquimedes Hidalgo García1, Janeth Villegas Guzmán1, Angela Chuquihuara Rodriguez1, César Cefferino Hidalgo1, Delinda De la Cruz Albújar1, Joel Peláez Troncos1, María Pacheco Alfaro1, Diana Yactayo Silva1, and Mayra Paredes Ballena1. 1Hospital Nacional Dos De Mayo, Lima, Perú.

Objectives: To describe a case of severe acute pancreatitis as a manifestation of systemic lupus erythematosus (SLE).

Methods: Clinical case report and literature review.

Results: A 24-year-old woman with SLE, grade IV nephritis and optic neuromyelitis under treatment with cyclophosphamide, presented to the emergency department with a one-day history of epigastralgia, with and intensity 9/10, not relieved by analgesics and associated with vomiting and hyporexia. Denied: alcohol use, use of azathioprine or gallbladder lithiasis.

On physical exam she presented easy hair detachment, oral ulcers; her abdomen was distended, depressed and painful to palpation in epigastrium and right hypochondrium. Arthritis of hands. Leukocytes 1170, lymphocytes 386, platelets 123000, Hemoglobin 9, amylase 801 (<110), lipase 14424 (<300), CRP 177mg / L, C3, C4 decreased. Proteinuria: 2 gr/24hrs.

Abdominal ultrasound: gallbladder without stones, signs of pancreatic inflammation, free fluid in cavity. TEM abdomen: Signs of pancreatitis. Colangioresonance: Signs of acute pancreatitis, absence of choledocholithiasis. Severe acute pancreatitis was diagnosed in the ICU. She received five pulses of methylprednisolone, with favorable clinical and humoral evolution, reason why it was concluded that the cause was autoimmune.

Conclusions: Pancreatitis as a gastrointestinal manifestation of SLE has a frequency of 0.7 - 4%, in 22% it is the initial clinical manifestation. Despite its low frequency, in the presence of acute abdominal pain, it must be considered in the differential diagnosis; even more if there is lupus activity since it is associated with a high mortality. This manifestation responds well to high doses of corticosteroids, as evidenced in our case, where timely diagnosis and treatment led to the remission of pancreatitis.

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Arquímedes Hidalgo García1, Diana Yactayo Silva1, María Pacheco Alfaro1, Janeth Villegas Guzmán1, César Cefferino Hidalgo1, Angela Chuquihuara Chuquihuara Rodríguez1, Delinda De La Cruz Albújar1, Joel Peláez Troncos1, Mayra Paredes Ballena1, and Fiorella Vilca Salguero1. 1Hospital Nacional Dos De Mayo, Lima, Perú.

Objectives: To describe the presentation of lung cancer in a patient with systemic lupus erythematosus (SLE).

Methods: Clinical case report and literature review.

Results: A 54-year-old woman with a diagnosis of SLE for 8 years, without a history of smoking. Request a consultation because of the presence of pleuritic pain in the posterior region of the right hemithorax and dyspnea of 2-months in duration. Denied: fever, night sweats, weight loss. On physical examination in right hemithorax: decreased amplexation, decreased vocal vibrations in 1/3 medium and abolished in the lower 1/3, egophony in the lower 2/3, absence of vesicular murmur in the lower 2/3 with dullness on percussion Study of pleural fluid: leukocytes: 3120 cells / mm3, red blood cells: 250 cells / mm3, polymorphonuclear cells: 0%, mononuclear: 100%, BK and GeneXpert: negative. Tumor markers: negative. Chest x-ray film: pleural effusion and heterogeneous radio-opacity in the right hemithorax. Chest tomography with contrast: lung mass in the middle of the right lung measuring: 84x55x27 mm with mediastinal adenopathies, effusion and pleural thickening. Cytological study, cell block and immunohistochemistry compatible with pulmonary adenocarcinoma.

Conclusions: SLE is associated with an increased risk of several types of cancer, including the lung with a 1,62 times increased risk in SLE. A Colombian study found a frequency of 4.4% of diverse neoplasms in SLE, but not of lung.

Lung cancer in SLE occurs on average at 60 years of age, late in the course of SLE, is strongly associated with a history of smoking; the most common histological type is adenocarcinoma and in 85% of the cases they are in advanced stages III / IV, being associated with a poor prognosis.

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Valeria Rodríguez Cárcamo1, Nilmo Chávez1, Silvia Rivera1, Estuardo Anzueto1, and Vinicio Montúfar2. 1 Department of Rheumatology, Guatemalan Social Security Institute, Guatemala, 2Department of Nephrology, Guatemalan Social Security Institute, Guatemala

Objectives: Renal involvement in systemic lupus erythematosus (SLE) is one of the main causes of morbidity and mortality. The risk for progression to end-stage kidney disease (ESRD) secondary to lupus nephritis (LN) in native kidneys has been estimated to be up to 30%. The objective of this study was to analyze the long-term outcome of patients with NL who underwent renal transplantation from a single center in Guatemala.

Methods: This is a medical records review study that included all adult patients diagnosed as having SLE in whom ESRD was primarily caused by lupus nephritis, who required renal transplantation from January 2005 to December 2017 in the Guatemalan Social Security Institute. Medical records of hospitalizations and follow-up of LN patients with a kidney transplant were reviewedf. All patients met the criteria for classification of the American College of Rheumatology for SLE. The diagnosis of LN was based on the findings of the renal biopsy, by the International Society of Nephrology/Society of Renal Pathology (ISN/RPS) 2003 Classification system. To perform the analysis, demographic and clinical characteristics, histological and immunologic features, and treatments used in the cohort of SLE transplanted patients were evaluated. Patient and graft survival rates were calculated with Kaplan-Meier survival curves.

Results: A total of 13 renal transplants were performed in 13 patients diagnosed with LN, during the 12-year interval. Most patients were women (n = 9, 69%), with a mean age of 37.4 ± 10 years at the time of transplantation. Twelve patients (92.3%) underwent hemodialysis before renal transplantation and 54% had such treatment less than 3 years. The most frequent lupus nephropathy was type IV (62%). Among the comorbidities of patients who received a kidney graft, 54% had arterial hypertension. Four transplants were performed from deceased donors and nine from living-related donors. The patient survival rate was 100%, and graft survival was 84.6% at 1 year. Graft failure occurred in a total of 2 transplantations and the causes were chronic allograft nephropathy and acute rejection. Only 1 patient presented a recurrence of lupus nephritis, which was placed on rescue therapy with Rituximab, with a renal graft currently functional.

Conclusions: Renal transplantation is a method which can provide a long-term survival for patients with SLE and end-stage renal disease at the Guatemalan Social Security Institute, as reported in other studies in Latin America.

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Yurilis Fuentes-Silva1, Claudia Elera-Fitzcarrald2, Cristina Reátegui-Sokolova2, Soledad Ibañez3, Carlota Acosta4, María Linares-Contreras5, Bernardo Pons-Estel6, and Cristina Drenkard7. 1 Universidad de Oriente, Ciudad Bolívar, Venezuela, 2 Hospital Guillermo Almenara Irigoyen, Lima, Perú, 3 Sanatorio Güemes, Buenos Aires, Argentina, 4 Complejo Hospitalario Universitario Ruiz y Páez, Ciudad Bolívar, Venezuela, 5 Sociedad Venezolana de Reumatología, Caracas, Venezuela, 6 Centro Regional de Enfermedades Autoinmunes y Reumáticas (CREAR), Sanatorio Parque, Rosario, Argentina, 7Emory School of Medicine, Atlanta, USA

Objectives: The Latin-American population living with lupus is eager to learn about the disease and how to deal with a variety of illness-related challenges. We describe the methodology and impact of a Facebook (FB) resource that provides patient-centered education in Spanish through live interaction between members of the audience and Latin-American healthcare professionals with expertise in lupus.

Methods: FB/Hablemos de Lupus (Let’s Talk about Lupus) is part of a comprehensive online program in Spanish to educate Latin-Americans about lupus. While FB/Hablemos de Lupus delivers a continuous stream of educational resources, a team of rheumatologists addresses members’ questions and evaluates the audience education needs. In July 2017, we launched monthly live video chats (LVC) led by healthcare professionals with expertise in lupus. LVC include a short overview of the topic by the expert, followed by the audience-expert chat. Experts received personalized training on the use of the FB tool, and guidance to leverage the live questions as opportunities to educate without providing diagnosis or treatment recommendations. A LVC team assisted the experts by collecting and tracking the live audience questions in a shared document. We measured FB LVC metrics (number of shares, likes, comments; people reached; times content was displayed; total views; total views by age, sex and location).

Results: By November 2018, FB/Hablemos de Lupus has broadcasted 12 LVC. Topics were selected from the most common themes that emerged organically from the FB audience. Most LVC were led by rheumatologists from the Latin-American Group for the Study of Lupus (GLADEL) and lasted between 40 to 60 minutes. The overall live audience included 3,319 people across 12 LVC. Among them, 90% were female (63% aged 25-54; 27% aged 35-44). All Latin-American countries were represented, with Mexico, Argentina, Colombia, Chile and Perú as the top 5. The largest non-Latin American audiences were from Spain and the United States. Means of LVC impressions (times the content was displayed), people reached (people who watched 12 LVC) and LVC views (times the LVC was viewed) were 112717, 80248 and 17867, respectively. Mean shares, likes and comments were 447, 1709 and 587, respectively. Experts valued the experience and were willing to participate in further sessions.

Conclusions: FB/Hablemos de Lupus LVC engaged large Spanish-speaking audiences across all Latin-American countries and around the world, reaching out lupus patients and caregivers from both populated and remote places. The live interaction with Latin-American rheumatologists and other qualified healthcare professionals appears to be an appealing and cost-effective resource to educate and empower people living with lupus. Latin-American experts were highly satisfied with the experience and valued the potential benefits of providing education via Facebook live video chats.

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Felipe Mosquera Moyano1,5, Murielle Rondeau-Lutz2, Jean-Christophe Weber2, Gabriela Zambrano Sánchez4, and Ramiro Cevallos3. 1 Pontificia Universidad Católica Del Ecuador, Quito, Ecuador, 2 Nouvel Hôpital Civil, Strasbourg, France, 3 Clinique Ste. Anne (GHSV), Strasbourg, France, 4 Universidad Tecnológica Equinoccial, Quito, Ecuador, 5Hospital de Especialidades Carlos Andrade Marin, Quito, Ecuador

Objectives: Giant Cell Arteritis (GCA) and Polymyalgia Rheumatica are the most common inflammatory conditions affecting the elderly population all over the planet. Both diseases share similar clinical, demographic and pathological features and often overlap in the clinical setting. Our study aims to determine the characteristics of the patients with GCA or PMR admitted in the Clinique Ste. Anne and the NHC of Strasbourg during a ten-year period.

Methods: We performed a medical record review descriptive analysis of the medical records in both the NHC and the Clinique Ste Anne of Strasbourg during a ten-year period going from 2004 to 2014 in which we identified 145 medical records in which the diagnosis of GCA or PMR had been registered. We constructed a database collecting the information concerning the clinical, biological, and pathological features of every patient as well as the treatment options used. The database was tabulated in order to perform standard statistical measures.

Results: Sixty-eight patients met all the inclusion criteria for our study, 75% were female (n=51). The chief complaint cited as the reason for hospitalization was a form of malaise (defined in Medical French as “alteration de l’etat general”) in 49% of the patients. The most common clinical history was the presence of cardiovascular disease found in 62% of the patients. Physical examination showed that the most prevalent disturbances were found in the musculoskeletal system corresponding to 36% (n=21) of all the cases. PMR was diagnosed in 60% (n=41) patients, while classic GCA was diagnosed in 25% of the patients and a form with mixed features was identified in two cases. Fifty-eight of all patients underwent a temporal artery biopsy in which a histolopathological diagnosis of GCA was found in 40% (n= 21) of all biopsies regardless of their clinical diagnosis.

Conclusions: This study highlights the importance of GCA and PMR in populations of European descent. It is to be remarked the high prevalence of cardiovascular diseases present in our group of patients which further highlights the importance of the disease as a significant cardiovascular risk factor in the elderly.

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Alejandro Nitsche1, Vera Milovic1, Alejandro Requejo1, and Juan Real1. 1Hospital Alemán, Ciudad Autónoma De Buenos Aires, Argentina.

Objectives: There are no specific treatments for Systemic Sclerosis (SSc). Some immunosuppressive or biological therapies offer benefit in skin tenderness and organ involvement. Meta-analysis, randomized trials and systematic reviews have demonstrated effectiveness of autologous hematopoietic stem cell transplantation (AHSCT) in SSc. Nonsmoker patients with early, diffuse and rapidly progressive SSc with mild pulmonary or renal involvement had better long-term event-free survival and overall survival, significant improvement in modified Rodnan score (mRS), pulmonary functional stability and better quality of life than those treated with cyclophosphamide (CY). AHSCT related mortality is nearly 10% versus patients treated with CY during the 1st year.

Methods: Case: 62 years old male patient with diffuse SSc with a disease duration of 14 years and a previous and stable mRS of 10 points. In 2017: severe and rapid progression of skin involvement with a mRS of 38 points, massive face telangiectasia, worsening of digestive features and 8 kg of weight loss. Secondary disease was ruled out. We discussed two treatment options: CY or RTX versus AHSCT but we favored the last one although, our patient had a SSc of more than 5 years in duration.

Results: AHSCT procedure: 1- autologous stem cell (ASC) mobilization with 5 days SQ filgastrim, 2-ASC recollection by venous leucapheresis with CD34+ selection. 3-A total of 11x10/6/kg ASC were recovered, 4-the patient was then admitted to the Hospital for non-immunoablative regimen: thymoglobulin: 6.5 mg/kg and CF 200 mg/kg; 5 days. Prophylaxis with fluconazole, ciprofloxacin and acyclovir were initiated, 5-Two days later, ASC were administrated through a central venous catheter, 6-Filgrastim 5ug/kg was added and at the 3rd day the patient had severe pancytopenia. Seven days after ASC administration, red and white cells begun to recover as well as platelets. At the 12th day the patient was discharged with acyclovir and trimethoprim sulfamethoxazole. After 11 months, our patient recovered appetite and gained weight, mRS decreased to 9 points, digestive manifestations disappear and improved the quality of life.

Conclusions: To our knowledge, this is the first SSc patient treated with AHSCT in Argentina. To minimize the AHSCT related mortality risk we need to identify patients with the highest possibility to improve (diffuse and rapid skin involvement - mRs >15-20-, <5 yrs disease, without or mild systemic damage) and lowest risk of complications. An expert team with a rheumatologist, cardiologist, pulmonologist and transplantation physician in a specialized center is required. Patients with early, diffuse rapidly progressive SSc have increased disease related-mortality (30%-50% at 5 years). AHSCT is a valid therapeutic option that can reverse the course of disease by “immune system resetting.”

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Nadia Riscanevo1, Maria Haye1, Janet Flores1, Diego Baenas1, Verónica Saurit1, Cecilia Alvarez1, Alejandro Alvarellos1, Francisco Caeiro1, and Soledad Fiorentino1. 1Hospital Privado, Córdoba, Argentina

Objectives: Systemic sclerosis (SSc) is a connective tissue disease characterized by activation of the immune system involving T and B lymphocytes, microvascular changes such as Raynaud's phenomenon, abnormal capillary architecture, digital ulcers, extended fibrosis of skin and internal organs. We aimed to establish frequency, clinical pattern and pharmacological interventions in patients with SSc.

Methods: Patients who fulfilled the ACR / EULAR 2013 criteria for SSc were selected from the Rheumatology Department at Hospital Privado Universitario Córdoba/Argentina.

A descriptive and cross-sectional study was conducted including patients diagnosed with SSc between February 2012 and December 2017.

Patients were classified into the following groups: limited cutaneous scleroderma (lcSSc), diffuse cutaneous scleroderma (dcSSc) and systemic sclerosis sine scleroderma (ssSSc).

The clinical manifestations evaluated were: Raynaud's phenomenon (RP), gastroesophageal reflux disease (GERD), telangiectasia and digital ulcers (DUs).

Data on capillaroscopy were classified as scleroderma pattern absent or compatible.

Doppler echocardiography was performed for screening pulmonary arterial hypertension (PAH).

The presence of interstitial lung disease (ILD) was assessed using chest CT.

Results: 61 patients were included; 93% were female, the median age at time of diagnosis was 49 ± 16 years.

46 patients (75%) presented lcSSc, 10 patients (16%) presented dcSSc, 5 patients (8%) presented (ssSSc).

The clinical manifestations: RP 59 (97%), GERD 54 (88%), telangiectasia 40 (66%), DUs 26 (43%).

Capillaroscopy, 40 (66%) patients had a pattern compatible with scleroderma.

Echocardiogram was performed in 58 patients (95%), in whom 8 patients (13%) presented (sPAP) > 40mmHg.

Chest CT was performed in 39 patients (64%), in whom 16/39 (27%) had ILD.

Anti-centromere antibodies were found in 35/57 patients (63%), ANA nucleolar antibodies in 9/57 patients (15%), Anti-Scl-70 antibodies in 6/40 patients (15%) and Anti-RNP antibodies in 5/45 patients (11%).

Treatment: phosphodiesterase type-5 inhibitor in 22 patients (36%), calcium inhibitor in 18 patients (30%), mycophenolate mofetil in 17 patients (28%), endothelin receptor antagonist in 4 patients (7%).

Conclusions: The most common clinical pattern was limited cutaneous scleroderma and clinical signs were RP and GERD with female predominance. An association was found between scleroderma pattern in the capillaroscopy and the presence of telangiectasias (p=0.038) and digital ulcers (p=0.027). The most common treatment was phosphodiesterase type-5 inhibitor.

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Lucila Garcia1, Victoria Martire1, Adriana Testi1, and Garcia Mercedes1. 1Hospital San Martín La Plata, La Plata, Argentina.

Objectives: The aim was to investigate the cumulative survival of belimumab and to identify causes of drug discontinuation and associated factors in SLE patients.

Methods: An observational study was done in a Rheumatology Department. Medical records and drug infusion registry were used to obtain information on the patients. Inclusion criteria were SLE patients who fullfilled ACR 82/ SLICC 2012 classification criteria, older than 18 years of ageand who had received at least one dosis of belimumab in a period between January 2013 and August 2018. Parametric and non-parametric tests were used according to the type of variables. Variables related to the survival of Belimumab were analyzed in the Cox regression model and were used as time variables. The permanence of the drug as a dependent variable and different demographic, clinical or therapeutic factors as independent variables. P values less than 0.05 were considered statistically significant. Cumulative drug survival was evaluated by the Kaplan Meier curve.

Results: Fourty-one female SLE patients were included. Mean age of 43 years (SD±9.5) and mean disease duration of 16 years.

The most frequent manifestations for the use of belimumab were musculoskeletal and cutaneous disease, 46% and 34%, respectively. Belimumab was discontinued in 15 patients. Causes of discontinuation: Patient decision 26%, unavailability 33%, lack of response, pregnancy, neoplasm, adverse effects, remission and lost to medical follow-up 1%. No demographic, clinical and laboratory factors associated with drug discontinuation were found. Twenty-six patients continued on belimumab. Mean age of the first drug infusion was 40 years and median time of use was 12 months (QIR 8-25). The median cumulative drug survival was 57 months (CI 95% 16.5-97.4). Using multivariate regression Cox model, no association was found between drug discontinuation and other factors. Drug survival was 81%, 51%, 35% and 24% at 6, 12, 18 and 24 months.

Conclusions: Belimumab cumulative survival was 57 months. The main cause of discontinuation was drug unavailability followed by patient decision. No demographic, clinical and laboratory parameters were associated with drug discontinuation.

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Erika Palacios Santillán1, and Fernando Naranjo Santos1. 1Hospital Eugenio Espejo, Quito, Ecuador.

Objectives: Establish the prevalence of heart involvement in patients with SLE, with or without associated APS at the Eugenio Espejo Hospital during theperiod from January 2015 to December 2017 and determine its association with traditional cardiovascular risk factors and SLE’s own risk factors.

Methods: Observational analytical epidemiological case-control study that included 109 patients from the Internal Medicine service of EEH.

Results: Cardiac involvement defined through electrocardiography and/or echocardiography was present in 65 patients with a prevalence rate of 59.6%. The most prevalent cardiac manifestations were: cavities enlargement (70.7%), valvular alterations (58.4%), and in third place pericardial disease (55.3%). Thrombocytopenia as a manifestation of SLE with or without associated APS was associatedwith the presence of cardiac involvement (OR 3.69; IC 95% 1.14-11.89, p= 0.02). A relationship between the presence of anti-cardiolipin aPL and thrombocytopenia could also be observed (OR 5.04, IC 95% 1.61-15.79, p= 0.003). Cardiac involvement in SLE was more frequent in patients with hypertension (OR 2.42; IC 95% 1.06-5.54, p=0.03) and those who were receiving betablockers (OR 6.56, IC 95% 1.41- 30.38, p=0.007) and anticoagulants (OR 5.05, IC 95% 1.38- 18.48, p=0.008).

Conclusions: Thrombocytopenia, hypertension, betablockers and anticoagulants treatment were associated with the presence of cardiac involvement. Thus, an initial and individual stratification of the risk is needed by conducting echocardiography which constitutes a non-invasive method, adequate for detecting cardiac abnormalitieseven in asymptomatic patients; also traditional risk factors as well as those associated with the disease itself need to be considered.

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Elias Fernando Rojas Báez1, Marcos Aurelio Vázquez Báez1, Gabriela Avila Pedretti1, and Isabel Acosta Colmán1. 1Hospital De Clínicas San Lorenzo Paraguay, San Lorenzo, Paraguay.

Objectives: To determine remission/survival in patients with a diagnosis of lupus nephritis and factors associated with it.

Methods: Observational, longitudinal, analytical-cut study, which included patients with a diagnosis of lupus nephritis who achieved remission being followed at the Rheumatology service of the Hospital de Clínicas for a period of 3 years. To calculate the survival of the response to treatment, the Kaplan-Meier estimator was used, as well as the Cox regression model for the association analysis.

Results: A registry of 50 patients was obtained, 42 (84%) women and 8 (16%) men. The mean age was 29,44 years ± 9,48. The total response of lupus nephritis to induction therapy was observed in 84% of patients at 10,5 ± 12 months and relapse was observed in 32% at 22 ± 16.74 months. Of these 80% were women with an average age of 30,2 ± 9.7 years. Partial response was observed in 14%; 71% of them were women with an average age of 24,7 years. The reasons for relapse were treatment discontinuation, infection and irregular treatment in (8%, 8% and 6%, respectively). The median survival time of the induction treatment was 18,75 months. Regarding the factors associated with treatment survival, no significant differences were observed between men and women, the presence of anti-DNA antibodies, the use of methylprednisolone, immunosuppressive treatment and biopsy type.

Conclusions: A total nephritis response was found in most of the patients, with a mean treatment survival of 18.6 months; relapse was mainly due to treatment discontinuation and intercurrent infections. No association was found between treatment survival and other factors.

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Andrea Cevallos1, Heidi Fernández1, Fernando Naranjo-Saltos1, Ruth Jimbo Sotomayor2, Diego Mera2, and Gabriela-Carolina Guevara1. 1 Hospital Eugenio Espejo, Quito, Ecuador, 2Pontificia Universidad Católica del Ecuador, Quito, Ecuador.

Objectives: Despite scientific advances, Systemic Lupus Erythematosus (SLE) continues to be an incurable disease with an unpredictable course that can considerably affect the health status of those who suffer from it and lead to disability. Therefore, since it is a chronic disease, problems surrounding the lack of treatment adherence and the evaluation of health-related quality of life (HRQoL) should be included in the state-of-the-art analysis for SLE.

Objective- Determine adherence to pharmacological treatment and health related quality of life in a group of 85 patients with SLE from Quito-Ecuador.

Methods: Prior authorization from the Human Research Ethics Committee for a descriptive, observational, cross-sectional study was obtained. The study was conducted in 85 patients with SLE at Eugenio Espejo Hospital in Quito-Ecuador. Sociodemographic and clinical variables of interest were collected and subsequently, treatment adherence with the Morisky scale was determined and the EuroQol 5D questionnaire for assessment of HRQoL was applied.

Results: The average age of the participants was 34.1 years. 39 patients (46%) were diagnosed with the disease between 1-5 years before this study took place. 77 patients (91%) did not present disease activity. 48 patients (56%) had comorbidities. The prevalence of polypharmacy was 56%. 60 patients (71%) did not get sufficientphysical activity. 48 patients had low treatment adherence (56%), 19 (22%) medium adherence and only 18 (21%) had high adherence. Patients self-assessed their health status, measured by the visual analogue scale, with an average of 76.6/100.

35% of the patients reported some problems with mobility and 5% described themselves as confined to bed. The self-care domain was better preserved, 87% reported no problems. 46% reported some problems on usual activities and 2% were unable to undertake them. 57% of patients described moderate or severe anxiety/ depression. The most affected domains of HRQOL, were pain and discomfort: 8 patients rated their pain as severe.

Conclusions: The evaluation of treatment adherence and HRQoL should be implemented as a strategy for the integral management in SLE. Our study, a pioneer in the field, showed that only 21% of patients had high adherence to treatment and that more than 65% reported moderate or severe permanent pain.

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Florencia Rodriguez1, Anastasia Secco1, Antonio Catalan Pellet1, Natalia Herscovich1, Cristina Amitrano2, Cecilia Asnal2, Alejandro Nitsche2, Julia Demarchi3, Damian Duarte3, C Segura3, Francisco Caeiro4, Nadia Riscanevo4, Veronica Saurit4, Silvia Papasidero5, Carla Gobbi6, Paula Alba6, Laura Raiti7, Veronica Cruzat7, Lida Santiago8, Sofia Velez9, Gabriela Salvatierra10, and Vicente Juarez11. 1 Hospital Bernardino Rivadavia, Capital federal, Argentina, 2 Hospital Alemán, Buenos Aires, Argentina, 3 Hospital Británico, Buenos Aires, Argentina, 4 Hospital Privado De Córdoba, Córdoba, Argentina, 5 Hospital Tornú, Buenos Aires, Argentina, 6 Sanatorio Allende, Córdoba, Argentina, 7 Clínica Bessone, Buenos Aires, Argentina, 8 OMI, Buenos Aires, Argentina, 9 Consultorio Privado S.V, Salta, Argentina, 10 IPRI, Santiago Del Estero, Argentina, 11Hospital Milagros De Salta, Salta, Argentina

Objectives: Primary Sjögren's Syndrome (pSS) is the connective tissue disease that is most frequently associated with other autoimmune disorders and approximately 30% of patients with SS have an associated autoimmune condition.

OBJETIVES: 1-Determine the frequency of patients who, following the diagnosis of pSS, developed another autoimmune rheumatic disease during their follow-up using the GESSAR database (Sjögren Syndrome Study Group of the Argentine Society of Rheumatology).

2-Detail the connective tissue diseases that occurred most frequently in these patients.

3-Describe the clinical, serological and salivary gland histology characteristics of these patients.

Methods: Descriptive, observational, cross-sectional study. We analyzed the data of patients diagnosed with pSS (American-European criteria 2002 / ACR-EULAR 2016), included in the GESSAR database. It was considered the development of another connective tissue autoimmune disease in those cases in which the diagnosis was added during follow-up.

Results: 681 patients were included, 94.8% were women, average age of 54(+/-14) and average age at diagnosis of 50(+/-13) years. 30(4.41%) developed another autoimmune rheumatic disease during their follow up. They were: Rheumatoid Arthritis 14, scleroderma 9, SLE 5, dermatomyositis 1 and rhupus 1. The mean age of this subgroup was 53(+/- 14), mean age at diagnosis of pSS 48(+/- 13). 91% were women. Regarding SICCA symptoms: 96% reported xerophthalmia, and 86.2% xerostomia. With respect to the objective tests: more than 80% had positive eye test, 81.2% positive sialometry, anti-Ro+ antibodies in 82.1% and anti-La+ antibodies in 33.33% of patients. 12/14 had a positive minor salivary gland biopsy. Prior to the diagnosis of RA, 78% presented with arthralgia and arthritis, 12 had RF+ and 2 anti-CCP+. Prior to the diagnosis of scleroderma, 44% had Raynaud's phenomenon and 22.22% were ANA positive with a centromeric pattern. Of the patients with the additional diagnosis of SLE, 1 had anticardiolipin antibodies and 100% reported arthralgia.

Conclusions: Of all the patients analyzed, 4.4% developed another connective tissue disease during their follow-up. We consider the importance of recognizing this possibility in order to arrive at an early diagnosis. The presence of certain clinical and serological manifestations could be considered as suggestive of the subsequent development of another autoimmune rheumatic disease.

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Victor Gustavo Martinez Aguero1, Gabriela Avila1, Marcos Vazquez1, Margarita Gonzalez1, Maria Teresa Filartiga1, Osmar Centurión1, Sonia Cabrera1, and María Isabel Acosta1. 1Hospital De Clínicas, Encarnación, Paraguay.

Objectives: To describe cardiovascular and non-traditional risk factors in patients with systemic sclerosis and to identify the association of these with the presence of atheromatous plaques at the level of the carotid intima.

Methods: An observational, prospective cross-sectional study with an analytical component, carried out during the period between April and August 2018 in the Rheumatology Department of the Hospital de Clínicas. The clinical variables of cardiovascular risk were recorded as sex, age, smoking, blood pressure, abdominal perimeter, body mass index, family history, use of corticosteroids, toxic habits, use of statins and immunosuppressants, in addition to the clinical involvement of patients. All of them underwent a carotid Doppler echo as a screening of the presence of atheromatous plaques at that level.

Results: A total of 47 patients were included, 11 (23.4%) of them were men and 36 (76.6%) women, the mean age recorded was 49.78 ± 15 years; at the time of the study, there were no patients actively smoking, 8 (17%) were ex-smokers, the systolic pressure had average values of 115.87 ± 18 mmHg and the diastolic of 72.39 ± 13 mmHg, the average abdominal perimeter was 87.16 cm ± 11.6. The average body mass index was 25.59 ± 4.4 Kg / m2, 20% presented antibodies to anti-SCL-70.

Doppler ultrasonography of the carotids was performed in 16 patients with atherosclerotic plaques in 7 (43.75%) patients, of which 3 had plaques with clinical significance as well as association with the use of NSAIDs (p: 0.033), presence of anti SCL-70 antibodies (p: 0.002), anti-centromere (p: 000); no associations were identified with the other variables.

Conclusions: In this cohort, the presence of traditional and non-traditional CVR factors was observed. The use of NSAIDs and positivity for anti-SCL-70 autoantibodies was found to be related to the presence of atheromatous plaques in carotids by carotid ultrasound.

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Goethe Salomón Sacoto Flores1, Luis Felipe Flores-Suárez1, and Natllely Ruiz1. 1Instituto Nacional de Enfermedades Respiratorias (INER) "Ismael Cosío Villegas", Ciudad De México, México.

Objectives: Case report. A 51-years old male was diagnosed in December 2015 with severe generalized granulomatosis with polyangiitis (GPA) due to ocular, ENT, pulmonary, musculoskeletal, renal, neurological, and cutaneous manifestations.

Methods: He was initially treated with methotrexate (MTX) 20 mg/week and oral prednisone (PDN) 1 mg/kg/day, achieving remission. One year later, while under MTX as maintenance treatment, he relapsed with identical symptoms. PDN 1 mg/kg/day and 6 intravenous cyclophosphamide (IV-CYC) pulses (1100 mg/pulse) were given. Despite treatment, pulmonary, cutaneous and renal symptoms persisted (BVAS 4).

Rituximab (RTX) 1g every two weeks for two doses was added with response. Cumulative PDN dose then was 14g. Nonetheless, five months later he developed new pulmonary nodules and active urinary sediment and was retreated with RTX as before. After improvement with lung nodules size reduction, they grew again 10 months later (BVAS 2). The decision was to combine both RTX and CYC as reinduction treatment.

Results: Two months later remission was achieved, and RTX has been continued for maintenance.

Conclusions: Combined treatment with RTX and CYC has demonstrated disease control in 96% of refractory cases at 6 months. The long-term evaluation of these strategies for both, induction and maintenance phases in conventional treatment-resistant cases, with the additional purpose to reduce the adverse effects by glucocorticoids, is still necessary.

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Goethe Salomón Sacoto Flores1, Melissa Guadalupe Rodríguez Gonzalez2, Natyelli Itzel Ruiz Gómez1, Daniela Peralta Charpenel1, Guillermo Perez Delgadillo1, Luis Humberto Silveira Torres2, and Luis Felipe Flores-Suárez1. 1 Instituto Nacional de Enfermedades Respiratorias (INER) "Ismael Cosío Villegas"., Ciudad De México, Mexico, 2Instituto Nacional de Cardiología Ignacio Chávez, Ciudad de México, México.

Objectives: Case description.

Methods: A 43-years old female presented to us with a history of pericardial tuberculosis (TB) which was treated satisfactorily in 2009. During diagnostic workup elsewhere, upper limb pulseless disease was noticed, and she was diagnosed angiographically with a Numano IIB + P Takayasu arteritis (TA) which was treated with methotrexate and prednisone. Remission was achieved in 2012. In 2017 she developed a saddle-nose deformity, nasal discharge and crusts, and in 2018 was seen by an ENT specialist who confirmed biphasic stridor and a 7 mm diameter subglottic stenosis (SGS) which led to laser dilatation. Biopsies obtained showed chronic inflammation and scarring, with negative search for microorganisms.

Results: This case could be labelled as limited GPA according to clinical findings and positive ANCA serology, although not PR3-ANCA as most frequently seen. However, more cases with limited upper airways involvement have been reported being MPO-ANCA positive. Treatment with MTX and low-dose PDN continues as maintenance, as no progression of TA and no recent activity of the probable GPA has been documented.

Conclusions: Six cases of coexisting granulomatosis with polyangiitis (GPA) and TA have been reported. However, none previously had SGS as GPA manifestation. SGS presents in up to 25% of GPA patients. TB might explain SGS due to scarring, but the rinosinusal features and the positive serology, with resolution of the pericarditis after specific treatment, -thought but not proven to be by M. Tb with positive cultures-, leads to think this infection might not be the culprit of this patient’s ENT symptoms.

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Patricia Sasaki1, Anastasia Secco1, Antonio Catalán Pellet1, Agustina Caceres1, Nadia Riscanevo2, Janet Flores2, Francisco Caeiro2, Cristina Amitrano3, Cecilia A. Asnal3, Alejandro Nitsche3, Carolina Segura Escobar4, Julia Demarchi4, Damian Duartes Noe4, Lida Santiago5, Laura Carmen Raiti6, Vanesa Cecilia Cruzat6, Carla Gobbi7, Silvia B Papasidero8, Sofia D. Velez9, Gabriela Salvatierra10, Vicente Juarez11, and Paula Alba7. 1 Hospital B. Rivadavia, CABA, Argentina, 2 Hospital Privado de Córdoba, Argentina, 3 Hospital Alemán, CABA, Argentina, 4 Hospital Británico, CABA, Argentina, 5 OMI, CABA, Argentina, 6 Clínica Bessone, Argentina, 7 Hospital Allende de Córdoba, Argentina, 8 Hospital Tornu, Argentina, 9 Consultorio Privado S.V, Argentina, 10 IPRI and Quorum Salud (Sgo del Estero), Argentina, 11Hospital Milagros de Salta, Argentina.

Objectives: Although there is little information, the debut manifestations of primary Sjögren's syndrome in adult patients 35 years of age and younger include less pronounced Sicca manifestations and a high degree of systemic involvement.

Objective: To describe and compare the systemic manifestations, assessed through the domains of the ESSDAI and the glandular manifestations, in Argentinian adult patients 35 years of age and younger and those older than 35 diagnosed with primary Sjögren's Syndrome.

Methods: Cross-sectional observational analytical study. We analyzed the data of patients older than 18 years, with diagnosis of primary Sjögren's syndrome (American-European criteria 2002 / ACR EULAR 2016), included in the GESSAR database (Sjögren Syndrome Study Group of the Argentine Society of Rheumatology), with minus one control in the last 12 months. The presence of systemic manifestations assessed from the domains of the ESSDAI and the glandular manifestations, and as possible confounders were considered: sex, treatments and time of follow-up in years of each group.

Results: 665 patients were included. One hundred with an age at diagnosis ≤35 years, 92% of them women. Within the glandular manifestations, statistically significant differences were found between patients ≤35 years vs >35 years, in xerophthalmia (90.72% vs 95.64%, p= 0.04) and xerodermia (42.35% vs 57.36%, p=0.03). No significant differences were found in xerostomia or xerovagina. Statistically significant differences were found in the following domains of the ESSDAI: peripheral nervous system (4.05 vs 11.32, p=0.03), respiratory (6% vs 15.40%, p=0.01) and renal (6% vs. 1.59%, p= 0.02). No statistically significant differences were found in the glandular, joint, cutaneous, central nervous system, hematologicalor biological domains. In a subanalysis of the ESSDAI domains, significant differences were found in arthritis (37.37% vs 27.16%, p=0.04) and persistent dry cough (4.04% vs 11.47%, p=0.03). No significant differences were found in: female sex, follow-up time, symptomatic treatment, treatment with pilocarpine, hydroxychloroquine, corticosteroids or immunosuppressants.

Conclusions: A significantly lower frequency of xerophthalmia and xerodermia was observed in the group ≤35 years of age compared to those >35 years. Regarding systemic activity, less involvement of the peripheral nervous system and pulmonary domain and higher in the renal domain, with statistically significant differences were found in those ≤35 years of age. These results suggest a lower glandular compromise in patients diagnosed at a younger age, without a characteristic differential pattern in terms of systemic involvement.

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Emelinda Tejada Reyes1, Ingrit Mercedes Nuñez1, Maria Marte Robles1, Rorayma Jimenez Candelaria1, Violeta Rosario1, Roberto Muñoz Louis1, Tirso Valdez Lorie1, and Rafael Alba Feriz1. 1Hospital Docente Padre Billini, Santo Domingo, Dominican Republic.

Objectives: To determine the relationship between the activity of the disease with the levels of vitamin D in systemic lupus erythematosus patients from the Hospital Docente Padre Billini, between August - October 2017.

Methods: This was a longitudinal and analytical study, where we determine the levels of vitamin D and calcium in SLE patients who attended the Rheumatology clinic of the Hospital Docente Padre Billini, during the period August - October 2017. The activity of the disease was ascertained with the SELENA-SLEDAI scale.

Results: The study population was constituted by 53 patients, of whom, 50 fulfilled inclusion criteria; 92% were female. The most frequent age range was between ages 31 - 45 years. 46% of the patients were ethnic mulatto. 54% had a disease duration of less than 5 years. The most used treatment was chloroquine in 94%, followed by Glucocorticoids 88%. Calcium values were normal in 82% of patients. The vitamin D values were insufficient in 66% and deficient in 18% of the study population. When using Pearson's correlation coefficient to determine if there was any relationship between vitamin D levels and disease activity according to SELENA-SLEDAI, we found that there was no relationship between these variables. The SELENA-SLEDAI was in low activity of the disease in 74% of the patients. Only 2% (1) had a high activity of the disease, which had deficient levels of vitamin D.

Conclusions: In this study, the activity of the disease was not related to decreased vitamin D levels.

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Candido Flores1, Rocío Chávez Mejía1, Guillermo Eduardo Ramírez García1, and Rafael García Rascón1. 1Hospital Regional De Alta Especialidad Ixtapaluca, Ciudad De México, México.

Objectives: Introduction: Rowell syndrome is a rare entity that occurs mainly in middle-aged women and is characterized by the association of systemic lupus erythematosus (SLE) and erythema multiforme (EM) type lesions, with positive immunological markers such as antinuclear antibodies (ANA), anti-Ro antibodies and rheumatoid factor (RF) positivity.

Objective: To present a clinical case of Rowell's syndrome.

Methods: Case: A 36-year-old woman, without a relevant past medical history. began her clinical picture 6 months prior to admission; she presented photosensitivity and erythematous, scaly and pruritic lesions located on the anterior aspect of the right forearm and in the occipital region; for this she received unspecified topical antimicrobial treatment on multiple occasions. Quantified intermittent fever up to 40 °C was noted as well as extension of the lesions to the periungual regions of both hands with extension into the palms; painful papules in the initially affected regions and in forearms, face, ears and scalp, some presenting a central necrotic appearance ("target" image) developed. Patient also developed interphalangeal arthritis and arthritis in elbows and knees. During follow up she presented ulcers in the fundus of both ocular sacs, anal region, vulvar and oral cavity. Patient was admitted to the hospital where a a diagnostc work up was initiated, positivity was determined for anti-ribonucleoprotein antibodies, ANA 1:640 homogeneous, C3 22, C4 1.66, ESR 46, anti-Ro 17.35, anti-La> 2, MPO> 2, PR3 4.88.

Results: Discussion: Rowell's syndrome is a rare syndrome, in which patients with SLE develop lesions similar to EM, it is associated with the presence of ANA with mottled pattern, positive RF and anti-Ro antibodies. A fundamental characteristic in the diagnosis of EM is the non-association with markers of autoimmunity. Cutaneous manifestations are varied and may include erythema, papules, target lesions, vesicles, blisters and urticarial lesions, with major distribution in arms and legs. Management is focused on SLE, with the use of steroids as the first line. The therapeutic response and prognosis is similar to SLE, with variable response and recurrences.

Conclusions: Although Rowell syndrome is a rare and controversial entity, it should be suspected in patients with SLE and EM-like lesions where there is no evidence of a precipitating factor. This patient fulfilled classification criteria for mucocutaneous SLE and for Rowell's syndrome, for which treatment was adjusted with oral prednisone and topical betamethasone, hydroxychloroquine, azathioprine, with remission of symptoms.

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Yessica Maria Ponce Delgado1, Ramiro Gómez2, Andrea Braillard2, Malena Viola3, María De la Vega3, Nicolás Pérez4, Graciela Gómez4, Amelia Granel5, and Óscar Rillo1. 1 Hospital General de agudos Dr. I. Pirovano, CABA, Argentina, 2 Hospital de Clínicas José de San Martín, Universidad de Buenos Aires, C.A.B.A., Argentina, 3 Hospital General de Agudos Dr. Cosme Argerich, C.A.B.A., Argentina, 4 Instituto de Investigaciones Médicas "Alfredo Lanari”, Universidad de Buenos Aires, C.A.B.A., Argentina, 5Hospital de Gonnet de la Plata, Provincia De Buenos Aires, Argentina.

Objectives: Targoff et al. reported the presence of Myositis Specific Antibody, "Protein Intermediary Factor" (Anti TIF-1) in patients with dermatomyositis (DM), associated with neoplasia and dysphagia and present in approximately 20% of patients with DM1.

MAIN OBJECTIVE: To describe the clinical characteristics of patients with inflammatory myopathies (MI) and the presence of anti-TIF 1- γ antibody, from the MII Registry of the SAR.

Methods: A descriptive medical records review study that analyzed the sociodemographic data, clinical characteristics and presence of anti-TIF1γ of patients in the MII registry of the SAR. Antibodies were studied by linear immunoassay (LIA) and the reagent was funded through a grant by the New Projects Committee (CONUPRO), managed by the MI Study Group (GESAR) of the SAR.

Results: We studied 71 patients from the Argentinian Registry of MII of the SAR, of which 7% (5 patients) were positive for anti TIF 1- γ, with a median of 65 (24-73) years, predominantly female (4/5). Ethnicity (2 Amerindians and 3 Caucasians). All presented the typical characteristics of DM. One patient had an associated neoplasia (breast cancer), poor clinical outcome and died. Three patients presented swallowing disorders. The presence of positive antinuclear antibodies (ANA) (mottled nuclear pattern 1/80) was observed in 2 patients. Four patients received immunosuppressive doses of glucocorticoids and immunosuppressants.

Conclusions: We found similar data to what has been previously reported in the literature; however, a higher frequency of dysphagia was observed (1). Only one patient had an associated neoplasm, which presented a poor outcome. Despite the small number of patients studied, this is the first report of patients from Argentina with anti TIF1 γ antibodies.

BIBLIOGRAPHY: 1. Ueda-Hayakawa, et al. Autoantibody to transcriptional intermediary factor-1β as myositis-specific antibody: clinical correlation with CADM or DM with mild myopathy. Br J Dermatol.2018; DOI: 10.1111 / bjd.17098.

2. Naoki Mugii, et al. Oropharyngeal dysphagia in dermatomyositis: Associations with clinical and laboratory features including autoantibodies. Plos One. 2016; 11 (5).

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Graciela Gomez1, Maria de los Angeles Gargiulo1, Amelia Granel2, Ana Marcos2, Ramiro A Gomez3, Andrea Braillard Poccard3, Malena Viola4, María Celina De La Vega4, Cecilia N Pisoni5, Micaela Cossati5, Mariano Rivero6, Mariana M Aciar7, Mariela Crespo7, Carolina Costi8, Mercedes A García8, Maria N Lojo9, Verónica Wernicke9, Belen Barrios10, Silvia Papasidero10, Paula Girard Bosch11, Rodrigo Garcia Salinas11, Jessica Ponce12, Oscar Rillo12, Dafne Capelusnik13, Isabel Pineda14, Roberto Movia15, Boris Kisluk16, Guillermo Berbotto16, and Susana Visentin17. 1 Instituto De Investigaciones Médicas Alfredo Lanari, Ciudad Autonoma De Buenos Aires, Argentina, 2 Hospital San Roque,Gonnet, La Plata, Argentina, 3 Hospital de Clínicas José de san Martín, Ciudad de Buenos Aires, Argentina, 4 Htal Cosme Argerich, Buenos Aires, Argentina, 5 CEMIC, Buenos Aires, Argentina, 6 Htal Británico, Buenos Aires, Argentina, 7 Htal Ntro Señor del Milagro, Salta, Argentina, 8 Htal San Martín, La Plata, Argentina, 9 Htal Rossi, La Plata, Argentina, 10 Htal Enrique Tornú, Buenos Aires, Argentina, 11 Htal Italiano, La Plata, Argentina, 12 Htal Dr. Ignacio Pirovano, Buenos Aires, Argentina, 13 Instituto de Rehabilitacion PsicoFisica, Buenos Aires, Argentina, 14 Consultorio Privado, Venado Tuerto, Argentina, 15 Hospital Evita, Lanus, Argentina, 16 Htal Eva Perón, Granadero Baigorria, Argentina, 17Htal Durand, Buenos Aires, Argentina.

Objectives: The diagnosis of Idiopathic Inflammatory Myopathies (IIM) may be a challenge for the Rheumatologist and complementary studies may not be available in daily practice.

The aim of our study was to describe the frequency of complementary studies performed in the diagnosis of patients with IIM from the Argentinian Registry and to compare between the present time and 2 years before.

Methods: Adults with dermatomyositis (DM) and polymyositis (PM), according to Bohan & Peter criteria, and inclusión body myositis (IBM) were eligible from the Argentinian Registry of Inflammatory Myopathies. Sociodemographics, laboratory and complementary studies were analyzed from available medical records. A commercial kit for the study of specific myositis antibodies (MSA), Euroimmun, was bought through a grant from the Argentine Rheumatology Society. Descriptive statistics, Fisher test(p<0,05). Stata v.11.0 was used.

Results: Two hundred and sixty-four patients were analyzed, mean age at diagnosis was 45 ys ±15, women n=180 (68%). Ethnic groups: Caucasian 42%, mestizo 35% and Amerindian 9%. The majority of the patients came from public and private hospitals from Buenos Aires City and La Plata; only a minority came from Salta and Santa Fe Provinces. The frequency of diagnostic procedures available was:CK in 254 patients (p)(96%), ASAT: 242p (92%), ALAT 239p (90%), EMG:170p (65%),RMI 170p (64%),muscle biopsy 108p (41%), CT: 215p(81%), DLCO 97p(37%), ANA:242p (92%),MSAs: anti-Jo-1 in 229 p (87%),anti-PL7,PL12, SRP and Mi-2 in 169 p (64%). Anti NXP2, OJ, EJ, TIF1, and anti-SAE in 60p (23%).

Sixty per cent of patients had health insurance. Frequencies in diagnostic methods were similar when they were compared with the first report of our Registry, although there was an increased in the access to MSA.Patients with health insurance had better access to muscle biopsy (p<0,001) and MRI (p<0,025) and electromyography (p<0,001).

Conclusions: - CK, ANA, anti-Jo-1 and Chest CT scan were done in more than 80% of the patients.

- Muscle biopsy was performed at a very low frequency: less than 30% of the patients.

- MSAs were available because of a grant from the Argentine Rheumatology Society.

- The accessibility to diagnostic techniques is heterogeneous and essential diagnostic procedures such as MRI, MSAs and muscle biopsy are difficult to obtain in our country.

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Yesid Jovanni Portilla Villarreal1, Ramiro Gomez1, Diana Dubinsky1, Sheila Leal Castro1, Diego Marino1, and Yuly Cuellar1. 1Hospital De Clínica San Martín, Caba, Argentina.

Objectives: To evaluate ocular toxicity from HCQ in patients with SLE

Methods: Our electronic SLE database (Jan 2014-June 2018) was searched. Patients fulfilling ACR 1997 criteria, withat least 1-year of follow-up, at least 12 week of HCQ treatment, who had a computerized visual field (CVF)study and who had discontinued HCQ due to retinal toxicity (RT) were included. Patients who had discontinued HCQ for other reasons (intolerance, hypersensitivity, muscular toxicity, skin rash) were excluded.

RT was defined as a decreased in visual acuity and/or worsening in CVF below reference levels that required HCQ discontinuation or dose adjustment.

Other possible causes of retinal abnormalities (diabetes, arterial hypertension), cumulative dose of HCQ at the time of its discontinuation, time of HCQ exposure and damage accrual as per the SLICC-DI at the time of HCQ discontinuationand at the last recorded visit were assessed.

Results: 231 patients were evaluated. 33 were included. 10 (30%) reinitiated treatment at a later time, in 5 of them with dose adjustment. HCQ discontinuation was definite in 23 (69.7%, 9.9% of the global population).

Patients with RT had mean age 44.5 (SD±12,6) years; Ethnicity: Amerindian 6,06%, Caucasian 6,06% and Mestizo 87,8%; 12 (36.3%) had arterial hypertension and 2 (6.06%) had diabetes.

Median cumulative dose of HCQ at the time of its discontionuation was 576 gr (IQR 144-1008). 9 (27.2%) patiens had a cumulative dose >1000 gr and 13 (39.3%) had >5 years of exposure.

Mean SLICC-DI at HCQ discontinuation was 0.8 (DS±1,2) and 1,17 (SD±1,7) at the last visit.

22 (70.9%) patients with CVF worsening were receiving HCQ >5 mg/kg (real weight) and 23 (74.1%) were receiving HCQ >6.5 mg/kg (ideal weight)

Year/Kg <1000 >1000

<5 20/33(60,6%) 0

>5 4/33(12,1%) 9/33(27,2%).

Conclusions: We observed a similar frecuency of HCQ discontinuation due to RT as reported in other studies. In our patients, there was no difference in RT between patients receiving <5 mg/kg (real weight) vs <6.5 mg/kg (ideal weight).

We found a higher frecuency of RT in patients with a cumulative dose of HCQ <1000 g and <5 years of HCQ exposure. These data may challenge the American Society of Ophthalmology’s recommendation of performing CVF after 5 years of exposure or >1000 g of cumulative dose.

An increment in the SLICC-DI score was observed after HCQ discontinuation, reaffirming the role of HCQ therapy in SLE.

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Mariela Mascote Márquez1, and Fernando Naranjo-Saltos1. 1Hospital Eugenio Espejo, Quito, Ecuador.

Objectives: To determine the initial clinical, immunological and epidemiological characteristics of 175 patients diagnosed with Systemic Lupus Erythematosus (SLE) of the Eugenio Espejo Hospital and to associate the SLEDAI-2K activity index at the onset of the disease with manifestations and complications.

Methods: Through an observational, descriptive and cross-sectional study, we analyzed the sociodemographic, clinical and immunological variables of patientsmeeting the 2012 SLICC criteria, being cared for by the Internal Medicine Service of the Eugenio Espejo Hospital during the 2015-2017 period. The variables were collected from the electronic file or system of medical records of the hospital, in a previously protocolized format, having been approved by the Ethics Committee in Human Beings Research.

Results: We studied 175 patients with SLE: 160 women and 15 men, with a female:male ratio 10.6:1, the average age of diagnosis was 29.3 ± 9 years, 87% were of mixed ethnicity. The most frequent clinical manifestations were hematologic 100%, musculoskeletal 69.7%, renal 56.6%, general 50.9% and mucocutaneous 47.4%. Deep vein thrombosis, haemoptysis and palpitations were frequent in men. 92% had ANA and positive anti-ds-DNA, followed by low C3 and anti-Sm with 64% and 57.4%, respectively. The mean SLEDAI-2K score was 11.7 ± 6.5 and 61.1% of the patients had severe activity at the time of diagnosis. There was an association between severe activity and proteinuria, lower limb edema, seizures, headache, arthritis, alopecia, leukopenia and lymphopenia. Nephrologic complications occurred in 56% of the patients (lupus nephropathy IV and III), followed by infections in 49.1% and neuropsychiatric manifestations in 22.9%.

Conclusions: The present study demonstrates how the clinical and immunological characteristics of this group of patients with SLE of the Eugenio Espejo Hospital are similar to those exhibited in other international studies. More than half of the patients had an elevated SLEDAI-2K index at disease onset, which translates into the severe involvement that patients receive from the health care systems in Quito-Ecuador.

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Hugo Javier Madariaga Charaja1, Rafael Salas1, and Ascuña Valery1. 1Hospital III Yanahuara EsSalud, Arequipa, Perú.

Objectives: To present the case of a diabetic patient with antibodies against insulin who improved with adjuvant immunosuppressive therapy.

Methods: Case report.

A 57-year-old woman, type 2 diabetic, under treatment with insulin lispro associated with NPH insulin for 3 years. Because of poor glycemic control she was changed to basal boluses with insulin glargine every 24 hours and insulin glulisine 3 times a day. She maintained good glycemic control for two years, with periodic dose escalation. Empaglifocin was added. One year before presentation, and despite an adequate dose of insulin, glycemic levels did not go below 300 mg/dl. Anti-insulin and anti-GAD antibodies were dosed, being positive in 2.9 and in 0.3 respectively. We also studied thyroid profile, ANA, Anti-DNA, anti-Sm, Anti-Ro and Anti-La, results were normal and negative, respectively.

Results: Immunosuppressive treatment was started with Azathioprine 100 mg/day and Deflazacort 30 mg/day, but glycemia persisted high. Treatment was switched to methotrexate 22.5 mg weekly and deflazacort 30 mg/day (until its discontinuation in 4 months), in addition to insulin glargine associated with glulisine. Patient presented 3 episodes of hypoglycemia, so the dose of insulin had to be decreased, currently with good response, sustained glucose control in values [ZERO WIDTH SPACE][ZERO WIDTH SPACE]lower than 160 mg / dl for more than six months.

Conclusions: - In patients with diabetes treated with insulin who do not achieve a good control the disease despite adequate insulin dosage, it is important the consider the presence of anti-insulin antibodies.

- Use of immunosuppressive therapy proved to be effective in this patient with diabetes mellitus treated with insulin who developed anti-insulin antibodies.

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Hugo Javier Madariaga Charaja1, Valery Ascuña1. 1Hospital III Yanahuara EsSalud, Arequipa, Perú.

Objectives: To describe the clinical-epidemiological characteristics of patients with a recent diagnosis of Ig G4-related ocular disease (IgG4-ROD).

Methods: All patients diagnosed for the first-time with Ig G4-ROD and who were cared for at the rheumatology and ophthalmology services of Hospital III Yanahuara, Arequipa- Perú, over the last two years were included.

Results: A total of 6 patients were included, 5 (83%) were females, median age at presentation 44 ± 14 years, minimum age atpresentation 29 years and maximum 77 years. The median time of illness prior to diagnosis was 0.7 months. 100% presented proptosis, restriction of eye movements, conjunctival hyperemia and decreased visual acuity, which was bilateral in 2 patients. The diagnosis was made by elevated serum Ig G4 in all patients and histopathological confirmation in 4 (67%). All patients had excellent clinical response to systemic steroids, 1 patient received treatment with methotrexate and 5 patients with rituximab. In all patients, proptosis resolved, and visual acuity wasrecovered.

Conclusions: - Orbital inflammatory disease is an ocular manifestation of Ig G4-related disease, being a frequent manifestation in our series with respect to other series.

- The response to immunosuppressive treatment is effective and prompt, especially with rituximab.

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Hugo Javier Madariaga Charaja1, Maria Elena Luza1, Carola Cervera1, Brissette Soto1, and Valery Ascuña1. 1Hospital III Yanahuara EsSalud, Arequipa, Perú.

Objectives: To describe the most frequent clinical manifestations and response to treatment of Ig G4-related disease in our environment.

Methods: All patients with the first-time diagnosis of Ig G4-related disease who were cared for at the rheumatology and ophthalmology services of Hospital III Yanahuara over the last four years were included.

Results: There was a total of 10 patients with this diagnosis. 80% were female. The most frequent clinical manifestation was Orbital Inflammatory Disease (60%) followed by Recurrent Parotiditis (40%). Ninety percent of patients had elevated IgG4 serum levels and histopathological confirmation in 40%, all of them with inflammatory orbital disease, the latter are classified as definitive cases, while the other 60% are classified as probable cases.

All patients had an excellent response to systemic corticosteroids and treatment with Rituximab (90%) and Azathioprine (10%). In 100% of patients with orbital involvement, proptosis resolved, and visual acuity improved. And in 75% of the parotiditis there was total resolution.

Conclusions: The most frequent presentation was the orbital. All had a good response to treatment with systemic corticosteroids associated with Rituximab in 90% and Azathioprine in 10%.

60% of cases could not be classified as definitive because there are still technical difficulties for taking a biopsy and there are no expert pathologists in our country to evaluate this disease.

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Hugo Javier Madariaga Charaja1, and Valery Ascuña1. 1Hospital III Yanahuara EsSalud, Arequipa, Perú.

Objectives: To present a case of inflammatory myopathy secondary to bevacizumab, because of its rare presentation.

Methods: Case presentation.

A 64-year-old woman diagnosed with cervical cancer, with metastases to the pelvis, stage III A according to IFGO (International Federation of Gynecology and Obstetrics), under oncological treatment of bevacizumab 1000 mg, carboplatin 400 mg, paclitaxel 200 mg for 17 months. In addition, medical history of motor sensitive axonal polyneuropathy in lower limbs treated with pregabalin 225mg / d for 15 months.

Results: She presents pain and progressive muscular strength reduction 4/5 of extensor muscles of the neck, thighs and calves. Total CPK: 1134, Electromyography informs Myopathy and muscle biopsy confirms inflammatory myopathy.

Conclusions: Bevacizumab is a drug widely used in cancer. In patients with pain and loss of muscle strength, although infrequent, inflammatory myopathy should be considered as an adverse effect.

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Hugo Javier Madariaga Charaja1, Maria Elena Luza1, Brissette Soto1, Carola Cervera1, and Valery Ascuña1. 1Hospital III Yanahuara EsSalud, Arequipa, Perú.

Objectives: To describe the clinical characteristics and therapeutic response of patients with a diagnosis of inflammatory myopathy refractory to treatment with Rituximab.

Methods: Three cases of patients with a diagnosis of inflammatory myopathy refractory to first-line treatment and with a good response to human immunoglobulin (IVIG), treated at Hospital III Yanahuara Arequipa-Peru over the last 4 years are included.

Results: Case 1: 57-year-old male with polymyositis, received treatment with Methotrexate and Rituximab plus steroids. CK persisted elevated from 310 to 490 u/L.IVIGwas indicated: 2 g/kg/month associated with prednisone 10 mg/day. From the fourth month until the present time CK has remained normal without the use of corticosteroids.

Case 2: A 52-year-old woman with autoimmune necrotizing myositis. Was treated with Methotrexate, and subsequently withRituximab plus steroids. CK persisted elevated from 670 to 978 u/L. Subsequently, IVIG 2 gr / kg was given monthly. At the second month of treatment, CK was 98; it has continued within normal limits until the present time.

Case 3: 46-year-old male with dermatomyositis. He was treated with Cyclophosphamide, Methotrexate, Azathioprine, Mycophenolate, Rituximab monotherapy and Rituximab plus mycophenolic acid and steroids. This treatment normalized the total CK for a few weeks and then the CK raised again (CK: 389-1025 u/L). IVIG was given 2 gr / kg monthly. At the third month the CK was 102 u / L, and it is still normal 6 months later.

Conclusions: - IVIG showed efficacy in achieving remission in patients with inflammatory myopathy refractory to conventional treatment and rituximab.

- If it was not for its high cost, IVIG could be the treatment of choice in cases of myopathies refractory to immunosuppressants, before Rituximab or in the future, it could become the treatment of choice.

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Karin Baumann1, Nelson Ortiz1, Johnatan Losanto1, Marcia Melo1, Sonia Cabrera1, Gabriela Avila1, Teresa Filartiga2, Ma. Isabel Acosta1, and Osmar Centurion1. 1 Hospital De Clínicas. Universidad Nacional, Asunción, Paraguay, 2Laboratorio Curie, Asuncion, Paraguay.

Objectives: Patients with Systemic Lupus Erythematosus have an increased cardiovascular riski due to the proinflammatory state of the disease which leads to increased morbidity and mortality.

Objective: Describe the cardiovascular risk factors in patients with Systemic Lupus Erythematosus.

Methods: Materials and methods: An observational cross-sectional study was conducted based on a survey in which cardiovascular risk variables were explored. The study included patients diagnosed with Systemic Lupus Erythematosus receiving their care at the Department of Rheumatology of the Hospital de Clínicas of the National University of Asunción from January to December 2018. The preliminary results of this project, supported by the Conacyt (l Prociencia 2015 grant, are now presented.

Results: Forty-three patients were included, of whom 95.3% were female and 4.7% male, with an average age of 31.7±9.4 years; 86% of the patients were from the metropolitan area; only of 35% of the patients had secondary education. Most patients (74%) were single; having 1.2 ± 1.4 children on the average.

Family history of hypertension was observed in 69.8%, diabetes 34.9% and dyslipidemia in 41.9% of the cases. Among the traditional risk factors were sedentarism in 74.4%, arterial hypertension in 27.9%, smoking in 11.6% and dyslipidemia in 9.3% of the patients. Patients had an average body mass index of 25.4 ± 5.8. Among the non-traditional risk factors renal involvement was found in 55.8% of the patients, antiphospholipid syndrome in 6.9% and the use of corticosteroids in 44.2% of the patients with a mean daily dose of 13.4mg.

As to the activity of the disease, patients had an average SLEDAI of 3.04±3.38.

Conclusions: Traditional and non-traditional risk factors were observed in patients with Systemic Lupus Erythematosus studied in the LUPUS PARAGUAY cohort. It is important to act in a timely manner on non-traditional cardiovascular risk factors and to create awareness in our patients of the importance of reducing traditional risk factors, so as not to add more cardiovascular risk than that of the activity of the disease.

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Sheyla Leal Castro1, Luis Orlando Roa Perez1, Sandy Sapag Duran1, Adriana Seewald1, Jaime Andres Betancourt Lopez1, Yesid Jovanni Portilla Villarreal1, and Diana Dubinsky1. 1Hospital De Clínicas José de San Martín, Ciudad Autónoma Buenos Aires, Argentina.

Objectives: Systemic Lupus Erythematosus (SLE) is an autoimmune disease in which inflammation is mediated by complement activation, immune complex deposition which lead to damage. Consumption of C3 and C4 complement fragments is accepted as a disease activity marker.

The aim of this study is to describe the clinical and serologic characteristics of SLE patients according to complement levels.

Methods: Our SLE electronic database was examined (Jan 2014-Aug 2016). We included patients ≥18 years, fulfilling ACR 1997/SLICC 2012 classification criteria, with at least one-year of follow-up and at least two complement levels determinations at different times during their follow-up.

Patients were classified in 4 groups, defined as following: Group 1 (normal C3 and C4 on all determinations), Group 2 (C3 and C4 below reference levels on all determinations), Group 3 (only C4 below reference levels on all determinations) and Group 4 (C3 and/or C4 on variable levels, with at least one determination below reference levels).

Demographic data, disease activity by SELENA-SLEDAI, damage accrual by SLICC-DI, presence of anti DNAds and anti Sm antibodies and clinical manifestations were assessed.

Statistical analysis was performed using Epi info v7. Correlation was assessed using χ or Fisher´s test as appropriate.

Results: 149 patients were included. 95.3% female, mean age at diagnosis was 30.6 years (CI: 28,5 – 32,8), mean disease duration 9.7 years. Mean SLICC-DI by group: Group 1: 1.0, Group 2: 0.5, Group 3: 1.3 and Group 4: 0.6. All groups with hypocomplementaemia showed a higher SLEDAI than Group 1 (p=0.0013).

Anti-dsDNA and anti-Sm were higher in groups with hypocomplementemia (p=0.01 and p=0.0001 respectively). As to the clinical manifestations, a significant difference was found in Lupus Nephritis (Group 1, 36.8%, Group 2, 44.1%, Group 3 78.6 % and Group 4, 36,4 % (p=0.0002)) and hemolytic anemia (Group 3, 28.6% and Group 1, 17.6 % (p: 0.0196)).

Conclusions: An association between C4 persistently below reference levels (Group 3) and anti-dsDNA antibodies and Lupus Nephritis was found. Group 3 patients may have a worse prognosis due to renal involvement.

Complement levels during follow up could be used as a marker to assess nephritis risk in SLE patients.

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Yurilis Fuentes-Silva1, Armando García1, Manuel Anés1, Irama Maldonado2, Carlota Acosta2, Luisa del Valle Ortega, Carlos A Rondón M3, Eylen Camargo4, Mayra Rauseo2, and Martín A Rodríguez5,6. 1 Universidad de Oriente, Ciudad Bolívar, Venezuela, 2 Complejo Hospitalario Universitario Ruiz y Páez, Ciudad Bolívar, Venezuela, 3 Hospital de Clínicas de Ceciamb, Puerto Ordaz, Venezuela, 4 Hospital Central de San Cristóbal, San Cristóbal, Venezuela, 5 Policlínica Metropolitana, Caracas, Venezuela, 6Centro Nacional de Enfermedades Reumáticas, Servicio de Reumatología, Hospital Universitario de Caracas, Caracas, Venezuela.

Objectives: Over the last several years many lupus patients in Venezuela have been with limited or no access to medications and under poor healthcare conditions due the current humanitarian crisis. The shortage or total absence of antimalarials and corticosteroids and the majority of immunosuppressants have been going on for quite some time but getting even worse since 2018. The regular use of medication is of paramount importance for an adequate control of disease activity and prevention of organ damage in lupus patients. In this study we have evaluated remission and LLDAS in Venezuelan patients with SLE and examined their correlation with medication availability and compliance.

Methods: From August to December 2018, 216 patients with SLE were evaluated; all patients fulfilled the 1997 ACR or the 2012 SLICC criteria. All patients signed an informed consent. We collected socio-demographics and related disease data: age, gender, education, occupation, disease duration, SLEDAI, SLICC-Damage Index. The remission state was assessed by the Definition of Remission in SLE (DORIS), LLDAS and non-optimally controlled (NOC) patients. We used dichotomous variables for medication compliance. Non-compliance time was investigated (< 3 months and ≥ 3 months). Statistical analysis was done using Chi square, Fisher´s exact test and Student's t-test for dichotomous and continuous variables, respectively. P-values of < 0.05 were considered statistically significant.

Results: 216 patients with SLE were included; 94% were female, mean age was 40.3±13 years, mean disease duration was 9.6±7.9. Patients mean education (years) was 13.1±3.9. Mean SLEDAI and SLICC-DI were 3.2±5.1 and 0.4±1.1, respectively; patients fulfilled criteria for remission in 42%, LLDAS in 65% (N=140) and NOC in 35% (N=76). Patients have been prescribed antimalarials, corticosteroids, immunosuppressants and biological agents in 95%, 62%, 47% and 0.9%, respectively. Medication non-compliance ≥3 months was 75%, 70% and 34.7% for immunosuppressants, antimalarials and corticosteroids, respectively. LLDAS and NOC groups were comparable for gender, age, education, occupation and disease duration. Lupus activity and number of hospitalizations during the previous six-month period were both significantly higher in NOC vs. LLDAS group (p<0.00001 and p=0.0001, respectively). There was a lower antimalarial treatment compliance in the NOC vs. the LLDAS group (p=0.01).

Conclusions: A high proportion of patients were non-compliant to prescribed medications mainly due to their unavailability in our country. However, the proportion of patients in LLDAS was higher than those in NOC. It is possible that the accumulated benefit of a previous regular therapy may have protected patients in the last few years of medication unavailability. Further work is warranted.

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Yesika De León1, Adriana Solari1, Gabriel Maciel1, Sandra Consani1, Carolina Díaz, and Jorge Facal. 1Hospital Maciel, Montevideo, Uruguay.

Objectives: To describe the clinical characteristics, specific auto-antibodies pattern and morphological changes in nailfold-video capilaroscopy in ambulatory systemic sclerosis (SSc) patients from Maciel Hospital.

Methods: Descriptive, observational, cross-sectional study.

Information was obtained from clinical records of patients with the diagnosis of SSc according to the 2013 EULAR/ACR criteria being care for at the Autoimmune Diseases Ambulatory Clinic, Maciel Hospital, between 2001 and 2017.

Patients were classified according to their clinical manifestations.

Data were collected in an Excel spreadsheet and expressed in absolute values, percentages and means.

Sex, clinical spectrum, skin and visceral involvement, antibody profile and morphological changes in nailfold video-capillaroscopy were recorded. A restrictive respiratory defect was diagnosed with restriction pattern on spirometry, reduction in DCLO and typical images in High resultion Chest CT (HRCT). Pulmonary arterial hipertension was estimated by Doppler echocardiography.

Gastroesophageal involvement was considered in patients with gastroesophageal reflux symptoms and/or compatible findings in upper endoscopy, esophageal manometry or barium esophagram.

Results: 68 patients with SSc. 65 (95.59 %) women. 3 (4.41%) men. Median age at diagnosis: 48.2 years. 3 patients with incomplete records were excluded.

Raynaud’s phenomenon was the most frequent first symptom (73.5%).

Clinical spectrum: limited SSc 33 (48.5 %), diffuse SSc 13 (19.1 %), preSSc 14 (20. 5 %), SSc sine scleroderma 4 (5.8 %), early SSc 4 (5.8%).

Clinical involvement: sclerodactyily 45 (66.1 %), puffy fingers 5 (7.3 %), Raynaud’s 66 (97 %), digital ulcers 6 (8.8 %), pitting scars 9 (13.2 %), calcinosis cutis 2 (2.9 %), telangiectasias 32 (47 %).

Gastroesophageal reflux: 32 (47 %).

Respiratory involvement: pulmonar artery hypertension 9 (13.2 %), insterstitial lung disease 11 (16.1 %).

Renal involvement: scleroderma renal crisis 1 patient.

Auto-antibodies: 56/68 performed: Results: anticentromere: 21, Scl-70: 12, negative antibodies: 25.

Nailfold video-capillaroscopy: 43/68 performed, pathologic 37/43 (86%).

Conclusions: Limited SSc was the most frequent clinical presentation. We found similar clinical features and auto-antibody profile as described previously. The disease was more prevalent in females, with Raynaud’s phenomenon, skin and esophageal involvement as common manifestations.

Pulmonary involvement was frequent in our cohort (near 1/3 patients). Sclerodermic renal crisis was extremely uncommon (perhaps due to the use of angiotensine-converting enzyme inhibitor treatment).

We emphasize the importance of early diagnosis and close patients’ follow up. This should allow physicians to diagnose early visceral involvement and begin individualized treatment in order to improve the patient’s prognosis.

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Diego Andrés Marino1, Yesid Jovanni Portilla1, Ramiro Adrian Gomez1, and Diana Dubinsky1. 1División de Reumatología, Hospital De Clínicas "José De San Martín", Ciudad Autónoma de Buenos Aires, Argentina.

Objectives: Primary objective: describe the histopathological characteristics of temporal artery biopsies (TAB) performed in patients with clinical suspicion of Giant Cell Arteritis (GCA).

Secondary objective: describe the clinical characteristics of patients suspected of GCA in which TAB was performed, evaluate a possible association between histopathological characteristics and clinical characteristics in these patients.

Methods: Descriptive, historical study. TAB performed in our center, a terciary university hospital in Buenos Aires, to patients with clinical suspicion of GCA between May 30th, 2005 and Feb 13th, 2017 were identified from the records of the Pathology service and included in this report. Histopathological characteristics were examind.

The clinical records of these patients were reviewed and epidemiological data, clinical characteristics (systemic symptoms, polymyalgia rheumatica, cranial symptoms and ischemic symptoms), laboraroty data (hematocrit, haemoglobin, white blood cell count, platelet count and ESR) and glucocorticoid dose at the time of TAB were analyzed.

Statistical analysis was performed using Epi Info v.7.22. A p<0.05 was considered significant.

Results: 59 TAB were included. The mean length of the biopsied samples was 19,8±9mm. 90% of the TAB were unilateral and 85% of them were performed under glucocorticoid treatment (mean dose 46,7±26.9 mg/day).

In 20% of the TAB, the histopathological diagnosis was GCA. In those, inflammatory infiltrate 100% (12/12) with a lymphocytic predominance, giant cells 100% (12/12), internal elastic disruption 33% (4/12) and granulomas 16% (2/12).

Mean age at time of TAB 70±12 years; male:female ratio was 1:2.9. 33% (10/30) had TAB compatible with GCA (TAB+).

Systemic symptoms 43% (13/30), and cranial symptoms 60% (18/30). Comparing TAB+ and TAB- patients, systemic symptoms were present in 60% vs 35%, cranial symptoms in 80% vs 50%. None of these characteristics was statistically significant.

Comparing TAB+ and TAB- patients mean ESR was 82±39 vs 64±41 mm/h. None of these parameters was statistically significant.

No association was found between clinical characteristics and histopathological characteristics.

Conclusions: In our study, only 20% of TAB met the diagnosis of GCA. However, most patients were under glucocorticoid treatment at the time of TAB and the mean length of the samples was 19 mm.

We did not find any difference in clinical manifestations or laboratory parameters in TAB+ vs TAB- patients nor any association between clinical characteristics and histopathological characteristics.

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José Maximliano Martínez Pérez1, Rafael Lopez1, Andres Zuñiga1, Genesis Maldonado1, Mario Moreno Alvarez1. 1Hospital Luis Vernaza, Guayaquil, Ecuador.

Objectives: Mycophenolate mofetil (MMF) is frequently used as an alternative treatment for lupus nephritis (LN). The results of global studies suggest good response in Latin American patients. The main objective of our study was to evaluate the response to 6-months induction treatment with MMF in moderate to severe LN. Secondary objectives were the improvement of 24h proteinuria, renal SLEDAI (rSLEDAI), serum creatinine and serum albumin.

Methods: We performed an analysis of patients treated with MMF as induction treatment for active LN between January 2009 and March 2018 in the rheumatology service at a teaching hospital in Guayaquil, Ecuador. Patients without treatment adherence were excluded from the analysis. Lack of adherence was defined as not taking continuous medication for at least 2 weeks. Demographic, clinical, laboratory and renal biopsy data were obtained from the electronic clinical history (ECH). Good response to treatment excluded patients who died, those needing rescue therapy, those developing end-stage renal disease due to LN or those not achieving a a 25% decrease in proteinuria g/24 h at the end of the induction treatment.

Results: Twenty-six patients received MMF, 21 were included. 95.2% (20/21) women; mean age 31.18y; time elapsed from diagnosis to LN 0.9 years. 100% were Hispanic. At the beginning 71.4% had low C3, 61.9% low C4, 81% had positive anti-DNA antibodies; and the median eGFR 83.36 ml/min/1.73m2. The average dose of MMF was 1.5±0.40 g with a median treatment duration of 6.10 months. Primary objective was reached in 95.2% (20/21) (Cochran's Q 40.00, p <0.001).There was a decrease in rSLEDAI (10.47 ± 2.97 vs 3.61 ± 3.07; p <0.0001), proteinuria g/24h (2.22 vs 0.48 IR, p<0.0001), albumin (3.44±0,53 vs 3.96±0.30, p<0.0001) g/dl and C3 (59.00 vs 91.0, p<0.0046) mg/dl. eGFR was stabilized (83.36 vs 90.12, p=01452) ml/min/1.73m2. Prednisone dose was significantlyt reduced (29.97 vs. 12.85 p<0.0001) mg/d. Only 8 (38%) patients had a renal biopsy; three class III and IV each and two class V. Adverse events occurred in 8 patients (38,1%); 75% (6/8) UTIs, one pneumonia, one pulmonary TB and two herpes zosters.

Conclusions: In our study of Latin American patients treated with MMF doses for real-life LN induction therapy we were able to demonstrate a good response. The number of patients and the lack of access to renal biopsy limit this study but we believe it is important to show the real-life data of a hospital in Latin America.

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Luis Velásquez1, Jhirly Mercado1, Alberto Cabello-Castillo1,2, and Yurilís Fuentes-Silva1. 1 Universidad de Oriente, Ciudad Bolívar, Venezuela, 2Complejo Hospitalario Universitario Ruiz y Páez, Ciudad Bolívar, Venezuela.

Objectives: A predisposition for developing neoplasms has been described in patients with systemic lupus erythematosus (SLE). This could be related to the SLE pathological process and/or associated factors such as comorbidities, treatments and associated viral infections. The aims of this study is to report the frequency of both benign and malignant neoplasms in patients with SLE and to investigate the risk factors related to them.

Methods: From August 2016 to October 2018, 155 patients with diagnosis of SLE (according to SLICC 2012) were evaluated. We investigated socio-demographic data: gender, age, education, occupation, body mass index (BMI), smoking and caffeine habits, age at menarche, hormone replacement therapy (HRT), oral contraceptive (OC) use, family history of neoplasm. We also studied variables related to SLE and possible comorbidities such as SLE duration, comorbidities, all treatments for SLE used since its diagnosis. Patients were divided into two groups: those patients who developed neoplasms (NG) and those who did not or controls (CG). All patients signed an informed consent. Statistical analysis was done using Chi square, Fisher's exact test and Student's t-test for dichotomous and continuous variables, respectively. p-values of <0.05 were considered statistically significant.

Results: NG (N=31) and CG (N=124) groups were comparable regarding gender, ethnicity, BMI, education, smoking status, caffeine use, OC use, age at menarche, HRT, a positive family history of neoplasm, positivity of ANA and anti-DNA and complement levels. Patients in the NG group were significantly older than the CG (44.3±13.9 vs. 38.9±13.1, p=0.02). Disease duration was significant longer in the NG vs. the CG (15.5±11.5 vs. 9.1±7.6, years, p=0.0001). We observed neoplasm in 20% of patients, only 5% was represented by malignancies. Main malignancies were breast, uterus and ovarian cancer. Pack-years index and cumulative dose of corticosteroid were significant higher in the NG than in the CG (p=0.02 and p=0.001, respectively). Regarding SLE treatment, mycophenolate was prescribed more frequently in the CG than in the NG (88.7% vs. 11.3%, p=0.015). The diagnosis of neoplasms was done prior to SLE onset in 37.5%, at the time of SLE presentation in 12.6% and after SLE onset in 46.9%.

Conclusions: We observed certain risk factors for neoplasms in SLE population: advanced age, longer disease duration, higher pack-year index and cumulative dose of corticosteroids. The majority of neoplasms appeared after SLE diagnosis. The more frequent neoplasms in this population were on the breast and uterus. Mycophenolate mofetil presented a possible protective effect for the development of neoplasms; further studies on this topic are warranted.

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Cristina Reátegui-Sokolova1,2, Rocío Gamboa- Cárdenas1, Mariela Medina-Chinchón1, Francisco Zevallos1, Paola Zeña-Huancas1, Claudia Elera-Fitzcarrald1,3, Víctor Pimentel-Quiroz1, José Alfaro-Lozano1, César Pastor-Asurza1,4, Risto Perich-Campos1,4, Zoila Rodríguez-Bellido1,4, Graciela S. Alarcón5,6, and Manuel Ugarte-Gil1,3. 1 Hospital Nacional Guillermo Almenara Irigoyen, Lima, Perú, 2 Unidad de Investigación para la Generación y Síntesis de Evidencias en Salud - Universidad San Ignacio de Loyola, Lima, Perú, 3 Universidad Científica del Sur, Lima, Perú, 4 Universidad Nacional Mayor de San Marcos, Lima, Perú, 5 Universidad Peruana Cayetano Heredia, Lima, Perú, 6University of Alabama at Birmingham, Birmingham, Estados Unidos.

Objectives: To determine the factors associated with absenteeism (percentage of the time missed from scheduled work-time over the preceding 7 days, due to SLE), presenteeism (percentage of time from scheduled work-time where productivity was impaired while patient is at work, over the preceding 7 days, due to SLE) and overall work impairment (combination of absenteeism and presenteeism) in patients with SLE.

Methods: A total of 133 consecutive (1997 American College of Rheumatology (ACR) criteria) working patients with SLE were assessed between October 2017 and December 2018, using a standardized data collection form. Sociodemographic, disease and work-related variables were collected. Disease activity was ascertained with the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI); disease damage with the Systemic Lupus International Collaborating Clinics/ACR Damage Index (SDI); health-related quality of life was assessed with the LupusQoL and fatigue with the FACIT. Work Productivity and Activity Impairment (WPAI) was assessed with the respective questionnaire; absenteeism and presenteeism due to overall health and symptoms during the past 7 days were scored. Linear regression models were performed to determine the factors associated with absenteeism, presenteeism and overall work impairment. Potential factors included were age at diagnosis, gender, socioeconomic status, educational level, SLEDAI, SDI, FACIT and the components of the LupusQoL.

Results: The mean age at diagnosis was 32.2 years (11.8); 121 (91.7%) were female. Nearly all patients were Mestizo. Mean years of education was 14.1 (2.6). The mean disease duration was 11.9 (7.5) years. Mean SLEDAI was 2.9 (4.0), and mean SDI was 1 (1.4). The mean percent of time for absenteeism was 5.0 (12.9), 28.5 (26.4) for presenteeism, and 31.3 (27.2) for overall work impairment. In the multiple regression analysis, factors associated with absenteeism were disease duration (B=-0.34; SE=0.12; p=0.007); pain (B=-0.14; SE=0.06; p=0.046); intimate relationship (B=-0.07; SE=0.03; p=0.046) and emotional health (B=0.16; SE=0.06; p=0.006), with presenteeism were physical health (B=-0.43; SE=0.14; p=0.002) and FACIT (B=-0.87; SE=0.30; p=0.005) and with overall work impairment were pain (B=-0.40; SE=0.11; p=0.001) and FACIT (B=-0.74; SE=0.28; p=0.010).

Conclusions: A poor HRQoL and a higher level of fatigue were associated with a higher percentage of absenteeism, presenteeism and overall work impairment in SLE patients. Addressing the factors related to HRQoL and Fatigue may have significant impact on work performance among SLE patients.

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Marília Sampaio-Barros1, Adriana Bortoluzzo1, Henrique Carriçoda Silva1, Ana Paula Luppino-Assad1, Danieli Andrade1, Rosa Maria Pereira1, and Percival Sampaio-Barros1. 1Universidade De São Paulo, São Paulo, Brazil.

Objectives: The aim of this study was to describe the frequency of symptomatic osteoporotic fractures in a large Brazilian cohort of patients with systemic sclerosis (SSc) and to analyze the clinical and laboratory parameters associated with their occurrence.

Methods: Methods: This is a medical records review study analyzing the diagnosis of osteoporotic fractures in the setting of acute pain complaint referred by 700 SSc patients who were being care for at a single SSc outpatient clinic in Brazil from 2010 to 2018. There was predominance of females (87.4%), white ethnicity (65.9%) and limited SSc (51.3%). Clinical, demographic and laboratory data were obtained from an electronic register database. Univariate and multivariate analysis were performed to eamine the factors associated with the occurrence of fractures. Statistical significance was considered when p<0.05.

Results: Among 700 SSc patients, 83 patients (11.9%) were diagnosed with at least one symptomatic osteoporotic fracture. Patients with osteoporotic fractures, when compared with those without fractures, were older (67.72+11.94 vs. 54.12+13.69 years; p<0.01) and were also older at disease onset (50.98+12.51 vs. 41.20+13.92 years; p<0.01) and at diagnosis (53.22+13.10 vs. 34.34+14.02 years; p<0.01); they also had a longer disease duration (16.81+9.24 vs. 12.92+9.32 years; p<0.01). In the female patients, age at menarche and age at menopause were similar in the two groups. Osteoporosis (OP) was significantly associated with female gender (p=0.014), limited SSc subtype (p=0.049), calcinosis (p=0.007), gastric involvement (p=0.043), pulmonary hypertension (p=0.042), and cancer (p=0.016). Regarding the specific SSc autoantibodies, there was statistical trend between OP with fractures and anticentromere (p=0.079) and anti-Scl70 antibody (p=0.068). Multivariate logistic regression showed that female gender (OR=3.296; 95%CI 0.988-10.993; p=0.052), alder age at onset (OR=1.088; 95%CI 1.064-1.114; p=0.012) and longer disease duration (OR=1.088; 95%CI 1.058-1.119; p=0.014) were associated with the occurrence of symptomatic OP fractures.

Conclusions: The frequency of symptomatic OP fractures was high in a large Brazilian SSc cohort, affecting predominantly older female patients in the setting of specific clinical manifestations.

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Paula Alba1, Maria Mercedes Vigliano1, Carla Andrea Gobbi2, Carla Maldini1, Gustavo Pepe2, Marcela De Marchi1, Viviana Neme1, Eduardo Horacio Albiero1, and Marcelo Augusto Yorio1. 1 Catedra de Semiologia. UHMI 3 UNC, Cordoba, Argentina, 2Catedra de Clínica Médica. UHMI 3 UNC, Cordoba, Argentina.

Objectives: Background: Metabolic Syndrome (MS) is highly prevalent in rheumatic diseases and it is recognized as a new independent cardiovascular risk factor. Objective: to study the frequency of MS in patients with Primary Antiphospholipid Syndrome (APS).

Methods: We studied patients with APS according to the Sydney Criteria at the Rheumatology Unit at a Cordoba Hospital from May 2014 to July 2018; a control group matched for age and gender was also studied. Clinical and demographic data were analyzed. Sera samples were tested for lipid profile, glucose, insulin level (IN), TSH, free T4, uric acid, and autoantibodies. MS was defined by 3 different criteria: World Health Organization (WHO), Adult Treatment Panel III (ATPIII) and International Diabetes Federation (IDF). p < 0.05 was considered statistically significant

Results: 80 patients were included, 71 females, with a mean age of 36 years and 80 as a control group, 71 females with mean age of 34.5 years. Weight, abdominal waist (AW) and BMI were 70.87 kg, 91.81 y 26.78 in APS patients and 70.11 Kg, 85.32, 25.95 in the control group. (p=0.76, p=0.001, p=0.36). Glucose level CT, HDL, LDL, and TG were 100.8, 176.6, 57.16, 109.8, 103.4 in APS patients and 99.5, 188.4, 63.62, 115.6, 94.9 in the control group (p=0.43, p=0.02, p=0.002, p=0.25, p=0.43). 12.5% of APS patients had MS according to OMS criteria and 16.7% according to ATPIII and 17.5% according to IDF definition and 8.8% of control group had MS according to OMS criteria, 13.3% according to ATPIII and 10% according to IDF definition (p= 0.44, p= 0.20, p= 0.16)

Conclusions: A higher frequency of different components of the MS was found in APS patients and there is a tendency according to IDF definition. MS is a known risk factor for cardiovascular disease, morbidity and mortality in systemic autoimmune diseases. A tight control of this condition in the early stages of the disease should be recommended.

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Carolina Segura Escobar1, Pedro Arbey Quevedo Mayorga, Cecilia Zaffarana, Demarchi Julia, Andrea Gomez, Guillermo Bartel, Geoffredo Earsman, Silvia Papassidero, and Andres Caicedo. 1Hospital Británico De Buenos Aires, Ciudad Autónoma De Buenos Aires, Argentina.

Objectives: To determine the frequency of ANCA in patients with SLE and to investigate if there are differences in the activity of the disease and the accumulated damage between ANCA positive and vs. ANCA negative patients.

Methods: Observational cross-sectional study. We included patients diagnosed with SLE according to the SLICC / ACR 2012 criteria, aged ≥18 years, treated at two rheumatology services of Buenos Aires recruited in the period from February to August 2018, and in whom the presence of ANCA by IFI and from PR3 and MPO by ELISA were available. Sociodemographic, laboratory and disease characteristics were collected from the medical records. The Systemic Lupus Erythematosus Disease Activity Index (SELENA-SLEDAI) and Systemic Lupus International Collaborating Clinics / American College of Rheumatology Damage Index (SLICC) scores were assessed at the time of enrollment into the study. Statistical analysis: Descriptive statistics. Student’s t test, Mann Whitney, Chi square and Fischer exact test.

Results: Thirty-six patients were included. 30 (83.3%) were women, 6 (16.6%) were men. The median age at diagnosis was 41.1 years (SD: 13.83), the median disease duration time was 7.6 years (SD: 5.95). There were 4 ANCA positive patients (11.1%); ELISAs were performed in 3 of them all being positive for MPO, none for PR3. When comparing those ANCA (+) vs ANCA (-) patients: The median age in years was 46.25 (SD: 5.61) vs. 40.46 (SD: 14.46) p=NS; the median diseas duration time was 12.25 (SD: 4.64) vs. 7 (SD: 5.9) years, p=0.03; the median of the SLEDAI score was 6 (SD: 6.92) vs. 4.09 (DS: 5.77) p=NS. The median SLICC Damage Index was 1.5 (SD: 2.38) vs 0.25 (SD: 0.76) p=NS; CRP values were high in 3 of the 4 the ANCA group (+) (75%) vs 8 (25%) p=0.028.

Conclusions: -11.1% of the study population was positive for ANCA, all of them with specificity for MPO.

-The variables associated with ANCA positivity in the univariate analysis were longer disease duration and a higher frequency of elevated CRP.

-Although the SLEDAI and SLICC-Damage Index scores in ANCA (+) patients were higher but this difference was not statistically significant.

-No significant differences were found between the groups in terms of sex, ethnicity, comorbidities, renal compromise or antiphospholipid syndrome; likewise, there were no differemces in laboratory parameters such as: Complete blood cell count, complement, proteinuria, renal function, or the presence of antibodies to DNA, Ro, La, Sm, RNP or Antiphospholipid.

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María Cecilia Argento1, Maria Florencia Martinez1, Juan Pablo Ruffino1, Ariana Ringer1, Nadia Cuadranti1, Ignacio Rolla1, Carla Achilli1, Juan Vandale1, Martin Baldomir1, María Soledad Constantini1, María Noel Cortese1, Mariano Palatnik1, Julieta Milanesio1, Graciela Rombo1, Ruben Darío Cavoduro1, Marcelo Abdala1, and Liliana Monje1. 1Hospital Provincial Del Centenario, Rosario, Argentina.

Objectives: To describe the clinical, anatomopathological and therapeutic characteristics of patients diagnosed with lupus nephritis (LN). To identify rebiopsies, the reasons that lead to this practice and histological changes.

Methods: Observational, descriptive and medical records review study. Patients from the Centenario Hospital, Rosario, Argentina, diagnosed with LN by renal biopsy for a period of 39 years (1978-2017) were included. Information was obtained from a database. Clinical, histological and therapeutic data was analyzed. The WHO classification of 1975 modified in 1982/1995 and subsequently the ISN / RPS classification of 2003 were used to describe histological findings in renal biopsies.

Results: 108 patients were included (90% women,10% men) Mean age: 29 years. Clinical presentations: 60% arterial hypertension, 45% nephrotic syndrome, 29% nephritic + nephrotic, 17% asymptomatic urinary disorder (AUD), 8% nephritic syndrome and 2% rapidly progressive renal failure. LN types:11.1% type II; 9.2% type III; 62% type IV; 12% type V and 2.7% mixed III + V. 38 rebiopsies were obtained from 34 patients. There were histological changes in 37%: 4 (10.5%) progressed from non-proliferative to proliferative and 7 (18.4%) from proliferative to non-proliferative. Most frequent change: from type IV to V. Reasons for rebiopsy: 30 cases of nephrotic syndrome, 7 of nephritic syndrome and 1 of AUD. Induction treatment: Cyclophosphamide (CP), Mycophenolate mofetil (MMF) and plasmapheresis. Maintenance therapy: Azathioprine 51%, MMF 32.4% and CP 13.8%. Evolution: 41.6% presented some type of remission (30.5% complete remission), 23% chronic renal failure; 11% were transplanted, 3% required hemodialysis and 18.5% died.

Conclusions: LN is an important predictor of morbidity and mortality. The transformation of renal lesions from one histological class to another occurs between 10-50% of the cases. Despite new therapeutic options, remission is achieved in 50-70% of patients and 10-20% progress to terminal chronic kidney disease within the ensuing5 years. In our study, 31.5% required renal rebiopsy, with a histological change in 37% of them and 29% progressed from non-proliferative to proliferative forms and vice versa. Renal rebiopsy is invaluable to assess the courseof kidney lesions due to SLE and to define treatment.

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Irma Dolores Reyes Banegas1,2, José Anibal Chacón Súchite1,2, Eva Griselda García1,2, Roberto Carlos Morales1,2, Lisseth Angélica Rodas Escobar1,2, and Abraham García Kutzbach1,2. 1 Asociación Guatemalteca Anti Enfermedades Reumáticas (AGAR), Guatemala, Guatemala, 2Universidad Francisco Marroquín (UFM), Guatemala, Guatemala.

Objectives: To determine the health status and quality of life in patients with systemic sclerosis (SS). To describe the demographic features of patients with SS. To describe the capillaroscopic findings in patients with SS.

Methods: We perfomed a descriptive cross-sectional study in which we reviewed sociodemographic - clinical characteristics, and quality of life, using a validated instrument. We performed a capillaroscopy exam, scored the modified Rodnan, the HAQ- DI (Health Assessment Questionnaire Disability Index) in patients being cared for at the AGAR clinic from August 2012 to October 2018. Inclusion criteria: Patients with diagnostic criteria for Systemic Sclerosis according ACR and EULAR, aged 18 to 90 years. Exclusion criteria: Localized Sclerosis or morfea, sclerodermiform syndromes.

Results: Thirty patients were included, mean age 58 years. 97% were female, 77% were housewives. The SS subtypes were: limited SS 77%, and difuse SS 33%. We found 30% of Overlap: Sjögren syndrome in 20%, RA in 10%. The mean time of disease duration was 5 years. The current treatment of patients was: DMARDs 80%, NSAIDs 66%, Steroids 26%.

The musculoskeletal, mucocutaneous and gastrointestinal clinical manifestations were the most common findings. The Rodnan modified score showed a slight skin thickening in most cases 76.6%. The HAQ-DI score was 2.03 SD 0.68. 56% of the patients had moderate to severe functional impairment.

The capillaroscopic findings showed: sclerodermiform pattern 22 (73.3%), normal pattern 2 (13.3) and tortuous pattern 2 (6.6%)

Conclusions: This study shows the important role of evaluating n the quality of life through the HAQDI. Likewise, it demostrates the importance of knowin more in detph the variability on capillaroscopic patterns and their correlation with the modified Rodnan score. All these variables can be predictive of disabling impairment in patients with systemic sclerosis.

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Lisbeth Aguila1, Henrique Carriço da Silva1, Ana Cristina Ribeiro1, Ana Paula Luppino-Assad1, Danieli Andrade1, and Percival Sampaio-Barros1. 1Universidade de São Paulo, São Paulo, Brazil.

Objectives: To identify environmental factors in a large cohort of patients with systemic sclerosis (SSc) examining their clinical and laboratory presentation.

Methods: A cohort of 662 patients being cared for at a single Brazilian SSc outpatient clinic was examined regarding environmental factors, including organic solvents, silica, pesticides, silicone implants and cocaine. Clinical, demographic and laboratory data were obtained from an electronic register database.

Results: The cohort consisted in 662 patients, with a predominance of females (87%), white ethnicity (66%) and limited SSc (50.8%), with a mean age of 52.4 (+14.2) years; 28.6% had positive anti-Scl70 and 25.4% anticentromere antibodies (ACA). Of them, 70 patients (10.6%) had known previous exposure to environmental factors. Organic solvents (51.4%), silica (20%), silicone (12.9%) and pesticides (11.4%) were the most frequent exposures. The group with positive environmental exposures was more likely to be of males (40% vs 10%; p<0.001), of non-Caucasian ethnicity (47.1% vs 32%; p=0.014), being previous or current smokers (48.6% vs 32.3%; p=0.007) and had a shorter disease duration (11.6+9.8 vs 13.7+9.3 years; p=0.01). Most clinical features, including disease presentation, organ involvement, capillaroscopic and serologic findings were similar between the groups. Remarkably, the exposure group had a higher frequency of myopathy (25.7% vs 14.5%; p=0.015) and pigmentary disturbances (22.9% vs 13.5%; p=0.036). After multivariate analyses, only previous exposure to environmental factors (p=0.015) and shorter disease duration (p=0.007) remained associated with myopathy.

Conclusions: This study showed that SSc patients with previous exposure to environmental factors are demographically distinct, probably due to occupational exposure, and present a higher frequency of myopathy and pigmentary disturbances.

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Carlos Arteaga1, Nancy Barrera1, Natalia Prieto1, Yeimy Trujillo1, and Philippe Chalem1. 1Fundación Instituto De Reumatología Fernando Chalem, Bogotá, Colombia.

Objectives: Characterize a cohort of patients with primary Sjogren's syndrome in the center of Rheumatology in Bogota, Colombia.

Methods: All samples were processed for ANA and anti-dsDNA by Indirect Immunofluorescence assay (IFA) in the automated HELIOS system, then ELISA tests were performed in the automated SQ2 system. All the results were confirmed with a second specific test. The reagents were from AESKU DIAGNOSTICS.

Results: The clinical characterization included age, diagnostic time, gender, eye symptoms, Conjunctival tumor, Oral symptoms, xeroderma, and recurrent parotitis.

The prevalence was higher in women than in men. Disease duration was on average 65 months. The main symptom was the ocular manifestations of dry eye, followed by xerostomia; the main systemic involvement was pulmonary involvement, with the presence of isolated bronchiectasis. Only about half of the patients presented clinical arthritis. No patient was found to be anti-DNA antibodies positive. Most of the samples were positive for SSA antibodies with fine speckled pattern by ANA. The prevalence of Anti-Alpha Fodrin IgA antibodies was higher than IgG.

Conclusions: It is possible to make an association between clinical manifestations and serological profiles. In general, the presence of autoantibodies correlates with age of onset, female predominance, increased risk of organ involvement, and the presence of other antibodies.

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Natalia Andrea Perrotta1, Maria Jose Lopez Meiller1, Veronica Malah1, and Diana Dubinsky1. 1Hospital De Clínicas José De San Martín, Ciudad Autónoma De Buenos Aires, Argentina.

Objectives: Systemic lupus erythematosus (SLE) has a bimodal mortality pattern, first peak: 20 years (relapses and infections) and a second later (cardiovascular diseases).

Hospitalization rate varies between 8.6 and 18.9% per year, up to 56% in two years. Hospitalizations for SLE flare: 11% to 80.8% and infectious causes of hospitalization: 10.9% to 37%. Other causes: cardiovascular disease, venous thromboembolism, associated autoimmune diseases, morbidity associated with pregnancy, complications due to accumulated damage and adverse drug reactions.

In this work we study the causes of hospitalization of patients with SLE admitted to a hospital in the Ciudad Autónoma de Buenos Aires, Argentina.

Methods: Review all patients with SLE admitted to Hospital de Clinicas Jose de San Martin, Ciudad Autónoma de Buenos Aires, Argentina, between January 2014 and December 2017.

Inclusion criteria were ≥16 years at the time of hospitalization and patients meeting the SLICC 2012 classification criteria.

Patient demographics, cause for hospitalization and medication lists, SLEDAI 2K and co-morbid conditions.

Descriptive statistics were obtained using standard statistical methods, with means (S.D.) and median (I.Q. R.) used for continuous variables and percentages used for other variables.

Statistical analyses were done using Epi Info version Independent-samples t-test analysis was performed to identify differences in means for continuos variables, Fisher's exact test using two tailed was used to analyze two dichotomous variables. p<0.05 was considered statistically significant.

Results: 121 hospitalizations (72 patients). F:M ratio 5. 25/72(34.7%) patients had more than one hospitalization and 32/121 admissions had more than one cause of hospitalization: 164 reasons.

Average age at admission: 40 years (SD:16.43). Duration of the disease between diagnosis of SLE and admission median of 5 years (range 0-35 years). At the time of admission: median SLEDAI 5 (range 0-27).

Flare: 86(52.4%): renal (34,8%). Infection, 44/164(26.8%): urosepsis 12/44(27.3%), pneumonia 11/44(25%). Uncommon causes: 28/164 (17%). Cardiovascular diseases: 6/164 (4.96%). Patients hospitalized for flare compared with other causes were younger (mean 35 (S.D.13.9); p<0.0017) and had a shorter disease duration (median 3.5 (IQR:1-7); p<0.0017).

The duration of hospitalization was 16.52 days (S.D.15) in patients with flares vs 11.75(S.D.8.9) days for other causes; p<0.03.

Intensive care unit (ICU): 17/121 hospitalizations (14.05%), median hospitalization time was 24 days (IQR;14-30) compared to 10 days (I.R.5-15.5) for those who were not admitted to the ICU; p <0.008.

3(2.48%) died: sepsis, obstetric and cardiogenic shock.

Conclusions: The main cause of hospitalization was flare (52.4%) which is in agreement with the literature. These patients were younger with a shorter disease duration.

The number of hospitalizations for infections was higher than in developed countries. This difference could be explained: use of different prevention strategies, lower socioeconomic level, less access to health systems, different hospitalization criteria and ethnic factors.

Cardiovascular diseases have been a rare cause of hospitalization. Possibly due to our patients’ disease duration time; thus there is greater disease activity and use of immunosuppressants, less dyslipidemia and less accumulated time of corticosteroids use.

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Andrés Zúñiga Vera1,2, Marietrini Kelly García Peñafiel2, Génesis Denisse Mera Chapi2, Rafael López Martínez1,2, José Martínez Pérez1, and Mario Moreno Álvarez1,2. 1 Hospital Luis Vernaza, Guayaquil, Ecuador, 2Universidad Católica de Santiago de Guayaquil, Guayaquil, Ecuador.

Objectives: To describe the clinical characteristics of patients with Systemic Lupus Erythematosus (SLE) admitted to the Luis Vernaza Hospital (HLV), with emphasis on Intensive Care Unit (ICU). To relate the ICU admissions and mortality with the intensity of the flares by SLEDAI-2K (Systemic Lupus Erythematosus Disease Activity Index) score.

Methods: A cross-sectional study was conducted in patients admitted to HLV between 2015-2017. The protocol of the RELESSER (Spanish Rheumatology Society Lupus Registry) cohort was followed from health records. Descriptive analysis were used for demographic and clinical variables, Pearson correlation test was used to relate SLEDAI-2K with ICU admission and mortality.

Results: 83 patients with SLE were admitted. 88% were women, age ranged between 15 and 24 years. Main clinical manifestations were: serological (88%), hematological (87%), renal and articular (both with 76%). Renal manifestations: proteinuria and cellular casts (65%), lupus nephritis (54%) and hematuria and pyuria (53%), 22% of patients progressed to renal failure. Hematological manifestations: non-hemolytic anemia (61%), lymphopenia (59%), leukopenia (40%) and thrombocytopenia (34%). Serological findings were: low complement (71%), antiDNA (58%), ANA Western Blot (57%), and antiphospholipid antibodies (54%).13% had antiphospholipid syndrome. Severe manifestations in patients admitted to the ICU were: lupus nephritis (76%), thrombocytopenia and renal failure (52%), valvular dysfunction and pleuritis (41%), psychosis and cardiomyopathy (38%). 8% of patients died during their hospitalization; 86% from the the ICU. ICU admission and SLEDAI score had a correlation of 35% and demise with SLEDAI score a correlation of 19%.

Conclusions: The most common clinical manifestations were serological, hematological, renal and articular. There was a very low positive correlation between the SLEDAI score and mortality and a low positive correlation between admission to the ICU and the SLEDAI score.

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Rafael Ignacio López Martínez1, José Martínez Pérez1, Andrés Zúñiga Vera1, and Mario Moreno Álvarez1. 1Hospital Luis Vernaza, Guayaquil, Ecuador.

Objectives: The aim of this study was to examine the clinic characteristics, management and outcome of systemic lupus erythematosus (SLE) patients with neuropsychiatric manifestations (NPM).

Methods: A cross-sectional study was conducted. SLE patients admitted between 2014 and 2018 to a high complexity hospital because of a disease flare and who also presented related NPM were included. Demographics data, clinical presentation, treatment and laboratory variables were recorded. Descriptive statistics were performed for data analysis.

Results: Twenty SLE patients with NPM were studied, 90% were women, mean age 26,57 years, time from diagnosis to NPM 0,35 years. Clinical manifestations: Nephritis 90%, skin involvement 65%, lymphopenia 60%, arthritis 55%, serositis 55%, leukopenia 20%, alveolar hemorrhage 15%, thrombocytopenia 15%, oral ulcers 15% and AHA 5%. Laboratory: ANA 100%, anti-DNA antibodies 90%, low complement 95%. anti-Sm 40%, anti-Ro 25%, anti-La 10%, anti-RNP 30%. Mean SELENA - SLEDAI score was 26,20. NPM: seizure 70%, acute confusional state 45%, cognitive dysfunction 20%, psychosis 10%, peripheral neuropathy 10%, myelopathy 5% and CVD 5%. Abnormal EEG 60%. MRI was normal in 20%, 35% had small punctate hyperintense lesions (HL) and 45% had extensive HL. All patients received IV methylprednisolone; immunosuppresors 55% -CYC (10/20), MMF (1/20), RTX 45%, IVIG 20% and plasmapheresis 10%. Combination treatment was necessary in five cases. Mortality rate was 5%. Alive patients recovered their neurologic condition after 6 months of being discharged from the hospital.

Conclusions: NP involvement is an early SLE complication during the course of a highly active and generalized disease. Despite the severity of the NPM, the functional prognosis is good, and the mortality seems to be low, if a wide therapeutic arsenal, multispecialty staff and necessary infrastructure are available.

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Guillermo Pons-Estel1, Manuel F. Ugarte-Gil2, Guillermina Harvey3, Daniel Wojdyla3, Russel Griffin4, Verónica Saurit5, Enrique R. Soriano6, Eloisa Bonfa7, Loreto Massardo8, Mario H. Cardiel9, Luis M. Vilá10, Rosana Quintana1, Graciela S. Alarcón11, and Bernardo A. Pons-Estel1. 1 Centro Regional de Enfermedades Autoinmunes y Reumáticas (GO-CREAR), Rosario, Argentina, Rosario, Argentina, 2 Universidad Científica del Sur, Lima, Perú, 3 Universidad Nacional de Rosario, Argentina., Rosario, Argentina, 4 Department of Epidemiology, The University of Alabama at Birmingham, Birmingham, Estados Unidos, 5 Hospital Privado Universitario de Córdoba, Córdoba, Argentina, 6 Servicio de Clínica Médica, Instituto Universitario, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina, 7 Faculdade de Medicina, Hospital das Clinicas HCFMUSP, Universidade de São Paulo, São Paulo, Brazil, 8 Centro de Biología Celular y Biomedicina Facultad de Medicina y Ciencia. Universidad San Sebastián, Santiago, Chile, 9 Centro de Investigación Clínica de Morelia SC, Morelia, México, 10 Department of Internal Medicine, Division of Rheumatology, University of Puerto Rico, San Juan, Puerto Rico, 11The University of Alabama at Birmingham, Birmingham, Estados Unidos.

Objectives: Classifying patients as having SLE is critical for clinical trials and observational studies; they are frequently used in clinical practice for early diagnosis. The SLICC 2012 criteria are more sensitive but less specific than the 1982/1997 ACR criteria. The 2018 EULAR/ACR criteria require a positive ANA as the entry point; clinical manifestations are clustered into weighted domains to maximize the accurate SLE classification, particularly of early disease.

To identify the items of the clinical and immunological domains of the EULAR/ACR SLE criteria that differ in the time to criteria fulfillment when compared to the SLICC criteria in two multiethnic cohorts.

Methods: Patients from two multiethnic, multicenter cohorts, one from the US and the other from Latin America (LA) were included. For these analyses, EULAR/ACR items were evaluated to determine which clinical manifestations and/or laboratory parameters could be of help to achieve an earlier classification of patients. Categorical variables were compared using Chi-square or modified Fisher exact tests, as appropriate. The statistical analyses were performed using SAS software version 9.4.

Results: Five-hundred forty-two patients out of 640 from the US cohort and 978 out of 1047 from the LA cohort were included. Only 35(6.5%) and 6(0.6%) of the patients achieved the 2018 EULAR/ACR criteria earlier in the US and LA cohorts, respectively. In turn, about 118(21.8%) and 280(28.6%) of the patients achieved them later, while the majority of the patients attained them at the same time. US cohort patients who accrued the 2018 EULAR/ACR earlier were more likely to be African American and Hispanic (from Puerto Rico) and less likely to be Caucasian or Hispanic from Texas. They also had more often mucocutaneous manifestations, arthritis, pleurisy and renal involvement. In contrast, LA cohort patients achieving the same criteria earlier were more likely to be Mestizo than African-Latin American. These patients also had more frequently alopecia, acute cutaneous lupus, pleural/pericardial effusion, leukopenia, positive antiphospholipid, anti-dsDNA antibodies and low C3 and C4.

Conclusions: We found that only a small proportion of patients met the 2018 EULAR/ACR criteria earlier than the SLICC criteria with no clear demographic, clinical or laboratory features associated to the earlier identification of lupus using these new criteria. We found no advantage of the 2018 EULAR/ACR criteria over the SLICC criteria for earlier disease identification in patients from these cohorts.

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Guillermo Pons-Estel1, Manuel F. Ugarte-Gil2, Guillermina Harvey3, Daniel Wojdyla3, Russell Griffin4, Verónica Saurit5, Enrique Soriano6, Eloisa Bonfa7, Loreto Massardo8, Mario H. Cardiel9, Rosana Quintana1, Graciela S. Alarcón10, and Bernardo Pons-Estel1. 1 Centro Regional de Enfermedades Autoinmunes y Reumáticas (GO-CREAR), Rosario, Argentina, Rosario, Argentina, 2 Universidad Científica del Sur, Lima, Perú, 3 Universidad Nacional de Rosario, Argentina., Rosario, Argentina, 4 Department of Epidemiology, The University of Alabama at Birmingham, Birmingham, Estados Unidos, 5 Hospital Privado Universitario de Córdoba, Córdoba, Argentina, 6 Servicio de Clínica Médica, Instituto Universitario, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina, 7 Faculdade de Medicina, Hospital das Clinicas HCFMUSP, Universidade de São Paulo, São Paulo, Brazil, 8 Centro de Biología Celular y Biomedicina Facultad de Medicina y Ciencia. Universidad San Sebastián, Santiago, Chile, 9 Centro de Investigación Clínica de Morelia SC, Morelia, México, 10The University of Alabama at Birmingham, Birmingham, Estados Unidos.

Objectives: Identifying SLE patients is critical for clinical trials and observational studies. The SLICC 2012 criteria are more sensitive but less specific than the 1982/1997 ACR criteria. The 2018 EULAR/ACR criteria differ from the others as they require a positive ANA as the entry point; in addition, the clinical manifestations are clustered into weighted domains to maximize the likelihood of an accurate SLE classification

The objective of this study was to identify the distinct items of the EULAR/ACR SLE classification criteria that differ in the time to criteria fulfillment when compared to the 1982/1997 ACR criteria in two multiethnic lupus cohorts.

Methods: Patients from two multiethnic, multicenter cohorts, one from the US and the other from Latin America were included. For these analyses, the 2018 EULAR/ACR criteria items were evaluated to determine which clinical manifestations and/or laboratory parameters could be of help in achieving an earlier classification of patients. Categorical variables were compared using Chi-square or modified Fisher exact tests, as appropriate. Of note, not all manifestations were available in these cohort. The statistical analyses were performed using SAS software version 9.4.

Results: Five-hundred and fifty-eight patients out of 640 from the US cohort and 956 out of 1047 from the Latin America cohort were included. Only 41 (7.3%) and 71 (7.4%) of patients achieved the 2018 EULAR/ACR criteria earlier in the US and Latin American cohorts, respectively. In turn, about one third of the patients in both cohorts achieved them later. Patients who accrued the 2018 EULAR/ACR earlier were more likely to have high anti-dsDNA titers and less likely to have mucocutaneous and joint manifestations.

Conclusions: When both cohorts were taken into account, those patients who achieved the 2018 EULAR/ACR criteria earlier had a lower frequency of milder manifestations (like mucocutaneous and articular) and tended to have a higher frequency of anti-dsDNA antibodies, suggesting these criteria could be more useful in subsets of patients with more severe disease.

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Daniela Grünholz Gambi1, Eduardo Waisntein Gurovich1, and Jonathan Sapaag Kaminski2. 1 Hospital Militar De Santiago, Santiago, Chile, 2Clinica Davila, Chile.

Objectives: The associated ANCA vasculitis correspond to systemic vasculitis that classically affect the upper airway, lung and kidney, however they can affect any organ. The involment of de central nervous system is rare; in granulomatosis with polyangiitis is described inbetween 2-8% of the patients the clinical presentation can be very serious or lethal, so an early and accurate diagnosis reduces complications. The neurological manifestations most frecuently described are pachymeningits, vasculitis of cerebral arteries, hypophyfitis, opnic neuritis and sensorioneural hearing loss. Belox we describe seven cases that were presented between 2014 and 2018 in the reumathology service of highly complex hospital in Santiago, Chile

Methods: After obtaininig the informed consent and the appoval of the local scientific ethics committee, an analysis of the patients records was carried out; data were obtained on the clinical presentation and management.

Results: We managed to obtain the data of 7 patients, 3 men and 4 women, average age at diagnosis of 64 years (45-70), 6/7 patiens were PR3 positive and 1 MPO positive; the form of presentation was as follow: 2 casese of retroorbital mass, 2 cases of pachymeningitis, 2 casese of hypophysitis, 1 case of sensorimotor otic involvement. In 3/7 patients the first manifestation of the disease were CNS symptoms; in the other 4 patients the diagnosis of vasculitis had been made previously.

6/7 patients received tratment with rituximab as management of neurological manifestations with excellent clinical course, and 1 patient with periorbital mass had a good response to cyclophosphamide. Of the 7 patients, most received previous treatment with cyclophosphamide or methotrexate and the decision to start rituximab was due to lack of response to previous treatment.

Conclusions: The neurological manifestations of ANCA- associated CNS vasculitis are rare but can be fatal or cause many sequelae. To our knowledge there are only reports of cases described in the foreing literature, most cases reports are from Japan. As described in the literature, most patients were PR3+. ANCA- associated CNS vasculitis can ensue in patients already diagnosed or be the first clinical manifestation. Rituximab seems to be an excellent therapeutic tool in this group of patients.

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Paulo Roberto Donadio1, Maria Natália Marques dos Santos1, Luiz Felipe Moraes Schwerz Bonadiman Blanco1, Louise Ferreira Iunklaus1, Pedro Henrique Iora1, Beatriz Medeiros Gurgel1, Vinicius Luís de Freitas Souza1, and Leonardo Grande de Almeida1. 1Universidade Estadual De Maringá, Maringá, Brazil.

Objectives: Systemic lupus erythematosus (SLE) is an autoimmune disease of unknown origin characterized by the formation of antibodies and deposition of immunocomplexes in various organs. In Brazil, there are 8.7 cases per 100,000 people per year, more frequently affecting African American and Hispanic women aged 20-40. We conducted this research with the objective of describing and analyzing the clinical characteristics and the course of the disease in patients with SLE hospitalized during a 5-year period in a tertiary referral hospital.

Methods: The hospitalizations were analyzed from January 2013 to December 2017. Data on age, gender, disease duration, medications used, reason for hospitalization, time and outcome of hospitalizations, hemodialysis, were collected directly from the medical records of these patients. The Excel and RStudio programs were used for data analysis: the first one was used to tabulate collected information, the second one was used for descriptive statistics and mean difference test.

Results: 228 medical records of patients being cared for at the Rheumatology outpatient clinic during the mentioned period were evaluated. There were 112 hospitalizations in61 patients (59 women and 2 men), approximately 26.7% of the patients being followed up, with an average of 5.82 days of hospitalization. The hospitalizations of patients with SLE occur more frequently in female patients, of fertile age, with an average disease duration of 7,92 years, and average of 5,82 days of hospitalization. The major reasons for hospitalization were a flare of the disease and the presence of infection, highlighting urinary and pulmonary tract infections as the most prevalent sites, and the use of corticosteroids associated with higher readmission rates, associated with systemic arterial hypertension.

Conclusions: As presented in this study, we conclude that hospitalizations of SLE patients occur more frequently in female patients of childbearing age, with an average disease duration of 7.92 years and an average of 5.82 days of hospitalization. The major reasons for hospitalization were disease reactivation (flares) and the presence of infection, highlighting urinary and pulmonary tract infections as the most prevalent sites; the use of corticosteroids was associated with high rehospitalization rates, especially in the presence of systemic arterial hypertension.

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Paulo Roberto Donadio1, Paola da Costa Souza1, César Orlando Peralta Bandeira1, Ademar Lucas Junior, and Luciano H Scremim. 1Universidade Estadual De Maringá, Maringá, Brazil.

Objectives: Report a case of a patient with systemic lupus erythematosus (SLE) and secondary Antiphospholipid syndrome (APS), who developed amyloidosis. Amyloidosis is described as a complication of chronic infections, and autoimmune rheumatic diseases, especially rheumatoid arthritis. Localized forms of amyloidosis in the oropharynx, larynx, and trachea are usually unrelated to other chronic diseases.

Methods: Female, 43 years old. Diagnosed with Lupus 9 years beforeand secondary APS with history of 3 pregnancies (1 cesarean delivery, and 2 abortions - 20th week and 8th week) and deep venous thrombosis. ANA 1/640 homogeneus; Anti-DNA: 1/160; Lupus anticoagulant: 2.08; Anticardiolipin IgM: 60.2 MPL/ml. One year before presentation, she developed a persistent sinusitis, with sensation of pressure in the left ear. At presentation her nasal sinus examination showed lesion in the rhinopharynx and auditory tube on the left. Otoscopy: slight pallor and bulging tympanic membrane. Endotracheal intubation for tympanostomy revealed partial obstruction of the trachea. Bronchoscopy: submucosal lesion in the posterior subglottic region causing partial light obstruction. Biopsy of the affected area was: suggestive of amyloidosis. Histochemistry and Congo Red staining confirmed the diagnosis. Magnetic resonance images: left and subglottic parapharyngeal lesions with marked luminal stenosis, with flow voids compatible with calcifications, characteristic of amyloid protein deposition. Warfarin was restarted, and hydroxychloroquine and colchicine added, with good response.

Results: The association of amyloidosis with chronic rheumatic diseases, mainly rheumatoid arthritis, has been described less frequently, due to the earlier treatment and control of these diseases, with prevalence estimated between 1 and 2%/million inhabitants. Mortality is mainly related to renal and cardiac forms. Localized involvement of the upper airways is rarely associated with systemic diseases, with Sjögren's Syndrome being described more frequently; there is no consensus for treatment of this presentation, but colchicine can be used successfully.

Conclusions: The case reported of localized and clinically stable amyloidosis, was treated with colchicine. As she is a patient with SLE and secondary APS, with a history of miscarriages and a venous thrombotic event, she is also receiving treatment with warfarin and hydroxychloroquine. Medline and LILACS sources searches for this association were negative; thus, the authors believe that this may be the first case of this unusual association in the medical literature.

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Daniel Fernández-Ávila1, Juan Gutiérrez1, Julián Rondón1, and Catalina Villota1. 1Pontificia Universidad Javeriana - Hospital Universitario San Ignacio, Bogotá, Colombia.

Objectives: To describe the clinical characteristics of patients with ANCA positive vasculitis at the University Hospital San Ignacio between 2005-2017.

Methods: Descriptive cross-sectional study. Patient records were reviewed between January 2005 and December 2017. The records were obtained using search systems in patients in whom ANCAs were requested (DISEARCH, LABCORE), obtaining 2072 results. 106 patients met the criteria for classification of the American College of Rheumatology (ACR) for ANCA-associated vasculitis (AAV).

Results: 106 patients diagnosed with ANCA positive vasculitis. Average age 55 years (± 14.7). 48.4% were women. In-hospital presentation in 68.8%. The average number of hospital days of stay was 16.6 (± 12.22), 56 patients (52.8%) had granulomatosis with polyangiitis GPA, 45 patients (42.4%) had microscopic polyangiitis (MAP) and 5 patients had eosinophilic granulomatosis with polyangiitis EGP (4.8%). 37 patients (35%) presented with alveolar hemorrhage, 22 patients (20.7%) presented renal compromise with rapidly progressive glomerulonephritis. Central nervous system compromise was documented in 25 patients (24%). Regarding treatment, 97 patients (91.5%) received treatment with intravenous or oral corticosteroid, 66 patients (62.2%) received treatment with cyclophosphamide. Plasma exchange therapy was performed in 15 patients (14.1%). 44 patients (41.5%) required renal replacement therapy. The in-hospital mortality of this group of patients was 20.4%, with sepsis being the most frequent cause of death (55%).

Conclusions: In this study, the most frequent ANCA positive vasculitis was GPA. Multisystemic compromise was present in all patient groups, the most common being pulmonary and renal, mainly in patients with MPA, similar to that reported by Solans-Laqué et al in Spain. Within the demographic characteristics, the average age at the time of diagnosis was around 50 years, predominantly in women which is consonant with the data from the Mexican study by Hinojosa-Azaola et al. The most frequent cause of in-hospital mortality was sepsis. Clinical information of patients with AAV is presented in a reference hospital in Colombia.Data are concordant with the literature.

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Nicole Bitencourt1, Bonnie Bermas1, E. Blair Solow1. 1UT Southwestern Medical Center, Dallas, United States.

Objectives: Inflammatory myositis occurs in about 3-8% of patients with systemic lupus erythematosus (SLE). In patients with SLE and inflammatory myopathy, myalgia and Raynaud’s phenomenon are common. Muscle biopsies are often consistent with dermatomyositis-like findings. The goal of this study was to identify common serologic, clinical, and pathologic features of patients with overlapping SLE and inflammatory myositis.

Methods: A medical records review was performed of patients seen at a single center safety net hospital who had an encounter diagnosis of SLE by ICD-10 code [M32.x, also included M36.x] and a history of muscle biopsy, EMG (electromyography), or elevated creatinine kinase or aldolase. Diagnosis of SLE was confirmed by one author (NB) using the 2015 American College of Rheumatology (ACR)/Systemic Lupus International Collaborating Clinics (SLICC) criteria. Patients with inflammatory myositis were defined by either muscle biopsy or with creatinine kinase (CK) elevation with muscle weakness with or without muscle magnetic resonance imaging (MRI) consistent with myositis. Data on patient demographics, clinical and laboratory features were extracted following approval by the Institutional Review Board (IRB). Chi-square was used to analyze serologic and clinical features and compare patients with scleroderma features to those without them.

Results: Ninety percent of patients identified as having SLE and inflammatory myositis were female and 60% were of African descent. Average age at SLE diagnosis was 34 years (range 14 – 57). A positive RNP (present in 75% of patients) was the most common serologic feature present. Among those in whom it was checked, about half of patients (18 of 37) had a myositis specific or myositis associated antibody.

Sixteen patients had a muscle biopsy available for review. Non-specific inflammatory changes were the most commonly observed muscle biopsy feature, followed by tubuloreticular inclusions by electron microscopy. Autoimmune necrotizing myopathy was described in four patients.

Just over half (27 of 52) of the patients identified with an inflammatory myositis and SLE also had features of systemic sclerosis clinically. Arthralgias and inflammatory arthritis were the most common clinical feature found, followed by Raynaud’s phenomenon. A surprisingly large percentage (33%) had thrombotic events.

Conclusions: Features of patients with inflammatory myositis and SLE are described. About half of the patients with SLE and myositis have clinical features of systemic sclerosis. Inflammatory myositis among patients with SLE appears to occur more commonly among patients of African descent. Myositis related, and myositis specific antibodies are common in patients with SLE and myositis. Muscle biopsy among patients with SLE and myositis has a wide range of findings, including pauci-immune necrotizing myopathy, dermatomyositis features, polymyositis features, and neurogenic atrophy. Thrombotic events are common among patients with SLE and inflammatory myositis.

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Marwin Gutierrez1, Annamaria Iagnocco2, Chiara Bertolazzi1, Fausto Salaffi3, Jaime Mendoza1, Luis Rodriguez1, Carlos Pineda1, Denise Clavijo-Cornejo1. 1 Division of Musculoskeletal and Rheumatic Disorders, Instituto Nacional De Rehabilitacion, Mexico City, México, 2 University of Turin, Turin, Italy, 3Universita Politecnica delle Marche, Jesi, Italy.

Objectives: To investigate the validity of ultrasound (US) in detecting subclinical interstitial lung disease (ILD) in systemic sclerosis (SSc) and to determine its potential in disease progression.

Methods: 133 patients without respiratory symptoms and 133 healthy controls were included. Borg scale, Rodnan skin score (RSS), pulmonary auscultation, chest radiographs and pulmonaryfunction tests (PFT) were performed. An expert rheumatologist blinded to the clinical data performed the pulmonary US. To determine the concurrent validity high-resolution CT (HRCT) scans were performed. After the baseline assessment, the patients were followed every 3 months for 12 months.

Results: A total of 54 of 133 patients (40.6%) showed US signs of ILD in contrast to healthy controls (4.8%) (p=0.0001). The clinical and laboratory variables associated with ILD were anti-centromere antibodies (p=0.005) and the RSS (p=0.004). A positive correlation was demonstrated between the US and HRCT findings (p=0.001). Sensitivity and specificity of US in detecting ILD was 91.2% and 88.6%, respectively. A good inter-observer reliability was observed (k = 0.72).

In the follow-up, a total of 30 patients (22.6%) who demonstrated US signs of ILD at baseline, showed changes in the ILD score by US.

Conclusions: US showed a high prevalence of subclinical ILD in SSc patients. It demonstrated to be a valid, reliable and feasible tool to detect ILD in SSc and to monitor disease progression.

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Esther Casablanca Alarcón1. 1INMUNOMED S.R.L., Cochabamba, Bolivia.

Objectives: To associate the urinary levels of TWEAK, MCP-1 and serological markers with remission and reactivation in lupus nephritis

Methods: Eighty-five patients were included (22 controls and 63 cases), who signed their consent prior to study participation. The control group corresponded to patients without clinical and family history of autoimmune disease. The case group met at least 4 criteria of the American College of Rheumatology for lupus diagnostic. This group was subdivided into 4 subgroups according to the activity of the disease. The levels of anti-ds-DNA, C3 and C4 complement levels, and clinic story of patients evaluated the activity of the disease; qRT-PCR was employed to measure the urinary levels of expression of the cytokine TWEAK and the chemokine MCP-1. The levels of these molecules were associated with the serum values of serological markers commonly used in clinical follow-up.

Results: The mRNA urinary expression of TWEAK and MCP-1 was significantly increased in patients with active LN (p <0.05). TWEAK expression correlated positively with serum levels of anti-dsDNA, anti-nucleosome and β2-microglobulin (r = 0.414 p <0.001, r = 0.405 p <0.001, r = 0.427 p <0.001). The level of expression of MCP-1 was positively associated with TWEAK expression levels (r = 0.299 p = 0.031), serum levels of anti-ds-DNA and anti-nucleosome antibodies (r = 0.390 p <0.05); r = 0.376 p <0.05).

TWEAK and MCP-1 mRNA expression were compared in the different study groups. There were significant differences in the expression levels of TWEAK in lupus patients with active disease versus those who were in remission with 58.3% (group I) and 100% (group II); the average of expression for both groups being 6 times higher than in patients with SLE in remission.

Conclusions: The present study demonstrates that MCP-1 and TWEAK can be considered as useful biomarkers to measure LN activity. There were differences between the expression levels of TWEAK and MCP-1 in patients with SLE and active LN with respect to those who were in remission or who belonged to the control group. Therefore, TWEAK cytokine and MCP-1 chemokine correlate positively with those patients who have active disease confirmed with high levels of anti-dsDNA antibodies, which suggests worsening of the disease. The measurement of mRNA expression levels of MCP-1 and TWEAK are non-invasive methods for the evaluation of LN.

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Toni Homberg Von Thaden1, Erika Vera Pérez1, Edgar Cervantes Trujano1, Andaluz Consuelo Garza1, Julio Ayala Balboa1, Maricela Martínez Corona1, Martin Hernández Quiroz1, Sonia Mayra Pérez Tapia1, María del Carmen Jiménez Martínez2. 1 Instituto Politécnico Nacional, Ciudad De México, México, 2Universidad Nacional Autónoma de México, Ciudad De México, México.

Objectives: Clinical research requires thorough evaluation of adverse events in patients exposed to new pharmaceutical agents. The Dialyzable Leukocyte Extract Transferon (DLE-T) is a complex drug, being itsmain component a mixture of human leukocyte-derived peptides less tha 10kDa in size which induces IFN gamma and it is being investigated as an immunoregulator.

The purpose of this study was to determine the safety profile of DLE-T in patients with autoimmune disease who have been treated with DLE-T as an adjuvant and provide evidence for its efficacy in rheumatoid arthritis.

Methods: This was a longitudinal study which evaluated the occurrence of serious and non-serious adverse events during DLE-T use in patients with autoimmune disease. Patients received oral DLE-T in varying doses (depending on disease activity) as an adjuvant to standard treatment in an ongoing safety and efficacy trial (IC 12-003). Efficacy was evaluated in a subgroup of patients with rheumatoid arthritis by measuring the changes in the Spanish Stanford Health Assessment Questionnaire (HAQ) 20-item Disability Index Scale scores. The HAQ addresses 8 sections: dressing, arising, eating, walking, higiene, reach, grip and activities, and scoring within each section is from 0 (without any difficulty) to 3 (unable to do). In this subgroup, DLE-T was added to conventional treatment in a weekly oral dose of 2mg/5mL for 4-6 months. HAQ scores were obtained before and 4-6 months after DLE was added.

Results: Safety: We evaluated 2179 patients with autoimmune disease who used DLE-T as an adjuvant. 176(8%) patients presented non-serious adverse events during DLE-T use. No patients presented serious adverse events. The most frequent adverse event was fatigue in 43(1.9%) patients, followed by headache in 39(1.8%) and joint pain in 21(1%). Adverse events were more frequent in pediatric patients (13.3% of all children exposed) as compared to adults (7.9%). Efficacy: 24 patients (23 female) with rheumatoid arthritis, ages 54±11 years added DLE-T to conventional treatment. Conventional treatment included methotrexate (67%), azulfidine (42%), oral glucocorticoid (38%), hydroxychloroquine (38%), leflunomide (8%) in various combinations plus non-steroidal antiinflammatory drugs (NSAID) (88%). 2 patients (17%) used no disease modifying agents and only NSAID due to liver damage. Baseline disability scores were 1.69±0.78. After 4-6 months of DLE-T use, mean disability scores were 1.29±0.75, p=0.001. 18(72%) patients showed improvement in disability scores, 11(46%) decreased the disability level.

Conclusions: DLE-T is safe to use as an adjuvant in autoimmune disease, no single adverse event was frequent (≥2% of the exposed population). It may improve symptoms in rheumatoid arthritis, but further controlled studies are needed to confirm our results.

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Juan Camilo Díaz-Coronado1,2, Sebastián Herrera-Uribe1, Deicy Hernandez1, Laura Betancur-Vásquez2, Jorge Lacouture-Fierro2, Daniel Gonzalez-Hurtado2, Juanita Gonzalez- Arango2, Laura Uribe-Arango2, Maria Fernanda Saavedra-Chacón2, Santiago Monsalve-Yepes2, Sebastián Guerra-Zarama2, José David Serna-Giraldo2, Juan David López-López2, Julián Barbosa-Arana2, Maria Camila Soto-Osorio2, and Ricardo A. Pineda-Tamayo1. 1 Grupo de información clínica, Artmedica, Medellín, Colombia, 2Departamento de medicina interna, Universidad CES, Colombia.

Objectives: Systemic lupus erythematosus (SLE) is a chronic, recurring and multisystemic autoimmune disease. SLE patients have 5-6 times greater cardiovascular risk than the general population due to a higher prevalence of traditional risk factors, such as high blood pressure, smoking, obesity, and inflammatory phenomena secondary to the disease and corticosteroid use, with an accelerated atherosclerosis. Our objective was to describe the associated characteristics with cardiovascular risk in a cohort of SLE patients and to compare these with a subgroup that has had cardiovascular events such as acute myocardial infarction (AMI) and non-fatal stroke.

Methods: This is a cross-sectional study including 1175 patient registers, that fulfilled the ACR 1997 or SLCC 2012 classification criteria and were attended in rheumatologic institutions between 2015 to 2017, in the Colombian cities of Medellín, Bogotá, Pereira, Armenia, Manizales, and Montería. We describe de sociodemographic features and clinical characteristics including exposure risk factors. Consequently, we compared these aspects between patients who presented cardiovascular events with those who did not present them.

Results: 91% were women and the average age was 43.9. The prevalence of hypertension was 27%, active cigarette smoking 61% and 36% of the population were obese and overweight. The prevalence of cardiovascular events was 5.2%, AMI contributed with 3% and non-fatal stroke with 2.2%. When comparing patients with and without cardiovascular events it was found that the first group was7 years older on average and had a longer disease duration (p<0,001). The prevalence of hypertension was higher in the group with cardiovascular events and was 57% (p<0,001). This group also showed higher damage index scores, being SDI >=1 (10% vs 5,4%). The results of this study are similar to those reported in other cohorts, highlighting that hypertension and age as traditional cardiovascular risk factors. The natural course of the disease is that major risk factor may contribute to cardiovascular disease with persisting inflammation and endothelial injury

Conclusions: Cardiovascular risk factors are more frequent in patients with SLE than in general population. The course of the disease is a frequent risk factor to highlight in patients with SLE and established cardiovascular events. This fact emphasizes the importance of effective and timely intervention of the described risk factors.

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Sebastián Herrera1, Juan Camilo Diaz-Coronado1,3, Deicy Hernandez1, Yecenia Durango1, Marcela Posada2, Jorge Mora2, Mariana Montoya2, Mateo Calle Estrada2, Cristina Posada2, Juanita Montaño2, Mateo Calle Estrada2, Maria Camila Soto2, and Ricardo Pineda-Tamayo1. 1 Grupo de información clínica, Artmédica, Medellín, Colombia, 2 Hospital General de Medellín “Luz Castro de Gutiérrez”, Colombia, 3Universidad CES, Colombia.

Objectives: Antimalarials (AMs) are an essential part of the treatment of systemic lupus erythematosus (SLE). Although they are safe, they have adverse effects that sometimes force patients to stop them increasing the risk of relapse. The current guidelines for the screening of maculopathy by antimalarials (MAM) do not have a big participation of rheumatology groups, generating controversy about the impact of some of their recommendations

Methods: We obtained data from a cohort of 1209 patients with SLE, followed between the years 2015 to 2018. A comparative analysis of patients with MAM outcome was carried out with different exposure groups, through Chi square and U de Mann-Whitney, getting raw OR association. A model was constructed taking into account crude associations with p <0.05, those that met the criteria of Hosmer Lemeshow and those that due to biological plausibility and literature review were important such as: use of chloroquine (CQ), age, dose in mg/kg of AMs, SDI damage index >0 and total cumulative AM dose. Logistic regression was performed to obtain adjusted OR.

Results: We analyzed 1006 patients, 72.2% used CQ and 27.7% used HCQ, were detected 7% cases of MAM. 6.6% due to CQ and 0.5% to HCQ.

In unadjusted analysis the use of HCQ showed lower probability of developing MAM (OR 0.21 CI95% 0.09-0.49). The cumulative dose of AMs ≥5.1mg/kg/day increased the risk of maculopathy (OR 5.6 CI95%1,04 – 32). The low weight, renal failure and age greater than 60 years, did not show significant differences.

In the subgroup’s analysis, patients who used CQ and had a BMI <18.5, GFR <30ml/min and liver disease had a greater proportion of maculopathy than those who used HCQ (P<0.02).

The dose of CQ <3mg/kg/day has a lower proportion of maculopathy compared to HCQ, and as a cumulative dose <400gr OR 0.89 (CI95% 0.81-0.97).

In our results, the dose of AMs associated with MAM is higher than the upper limit proposed in current screening guidelines.

Conclusions: We suggest an overestimation of the association with retinal toxicity due to CQ. In some subgroups of risk, we found association with maculopathy (BMI, dose, use of CQ, liver disease, renal dysfunction) are similar to those usually described in the literature as predisposing to ocular toxicity.

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Paulo Roberto Donadio1, Felipe Merchan Ferraz Grizzo1, Lorena Bossoni Miosso Marchiotti1, Sueny de Paula Monarin1, Gabriel Bortoli Ramos1, Milene Cripa Pizatto de Araújo1, Rodolfo Grillo Menegon1, Vitor Corsaletti Abreu1, and Pedro Henrique G de Andrade1. 1Universidade Estadual De Maringá, Maringá, Brazil.

Objectives: Cryptococcosis is a fungal infection of a systemic and opportunistic nature that occurs through the inhalation of Cryptococcus spp, mainly Cryptococcus neoformans, that affects immunocompromised individuals, especially those with acquired immunodeficiency syndrome, and Cryptococcus gattii, that affects mainly immunocompetent individuals. In systemic lupus erythematosus (SLE) treated with immunosuppressants, cases of this fungal infection, usually by Cryptococcus neoformans, with a much lower frequency and more favorable evolution, without a significant increase in mortality, nor severe sequelae are also reported. The authors describe the case of a patient with SLE who presented fungal infection of the central nervous system by Cryptococcus gattii.

Methods: Female, 26 years old, with SLE and nephritis class IV, on treatment with monthly pulse of cyclophosphamide, third cycle on 03/04/2012, and prednisone 60 mg/day, presented a holocranial headache one week before presentation, nausea and vomiting; and 2 days before, fever (38°C - 40°C). Physical examination: no neck stiffness or focal deficit. Computed tomography of the skull showed an increase of basal cisterns, brain grooves and lateral fissures. No further changes. CSF showed 3 erythrocytes/mm3, 1277 leukocytes/mm3 (87% mononuclear), 182 mg/dl protein, glucose <20 mg/dl, lactate 47 mg/dl. India ink staining showed the presence of Cryptococcus sp. Cryptococcal antigen 1: 4096, VDRL and BAAR negatives. Serology for HIV negative. Culture for fungi showed growth of Cyptococcus gattii. Treated with Amphotericin for 21 days and Fluconazole for 48 days, evolved without sequelae

Results: With the evolution of therapy for SLE, the survival of patients with SLE has increased significantly; there has been also a reduction in mortality rates due to complications of the disease. In contrast, death rates from infections have increased; as to to CNS involvement, fungal infections have been reported as the most important ones, especially those caused by Cryptococcus spp. Cryptococcus neoformans has the characteristic of infecting immunosuppressed patients, while Cryptococcus gattii, which predominates in tropical countries, is more commonly associated with infections in immunocompetent patients.

Conclusions: The case described here can be considered a rarity, since there was culture evidence of Cryptococcus gattii, which, as far as we were able to verify, there is no evidence in the literature of Cryptococcus meningitis for this serotype in patients with SLE. The treatment instituted completely controlled the clinical manifestations leaving no neurological sequelae.

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Zahi Touma1, Dafna Gladman1, Kaiyin Fei2, Irene Gregan2, Federico Zazzetti3, Robert Gordon2, Kim Hung Lo2, Shawn Rose2, and Murray Urowitz1. 1 Krembil Research Institute, Toronto, Canada, 2 Janssen Research & Development, LLC, Spring House, United States, 3Janssen-Cilag, Buenos Aires, Argentina.

Objectives: While traditional Systemic Lupus Erythematosus (SLE) Disease Activity Index 2000 (SLEDAI-2K) scoring assesses complete SLE response for individual disease manifestations, the SLEDAI-2K Responder Index-50 (S2K RI-50) evaluates responses using partial improvement (≥50%) in each of the 9 organ-systems of SLEDAI-2K and generates a total score. We aimed to evaluate the performance of S2K RI-50 at 24 weeks in a randomized, placebo (PBO) controlled trial of UST in patients with moderate-to-severe SLE disease activity to ascertain a minimal threshold of partial improvement.

Methods: The UST phase 2, PBO-controlled study enrolled adults with active disease (SLEDAI score ≥6 with ≥1 BILAG A &/or ≥2 BILAG B scores) despite standard-of-care therapy. Patients (n=102) were randomized (3:2) to receive UST IV ~6 mg/kg or PBO at week (wk) 0, followed by SC injections of UST 90mg q8w or PBO beginning at wk8, both added to standard of care. We calculated S2K RI-50 response in patients receiving UST (n=62) vs PBO (n=40) at wk24 using increasing S2K RI-50 reductions of 1, 2, 3, 4, 5, or 6 points from baseline to determine ≥50% improvement. In order to determine a minimal cut-off to discriminate a treatment effect reflecting partial improvement, nominal α level was set at 0.05 for this post hoc analysis.

Results: The performance of S2K RI-50 in detecting the treatment difference between UST and PBO was examined. A 2-point reduction in S2K RI-50 was the lowest threshold to demonstrate a p-value <0.05 (response rates were UST (93.5%); PBO (79.3%); treatment difference 14.2%, p=0.03). As an example, this could reflect partial improvement in one of the following: renal, arthritis, or myositis, or partial improvement in two of the following disease manifestations: rash, alopecia, mucosal ulcers, pleurisy, pericarditis, low complement or increased anti-dsDNA.

Conclusions: S2K RI-50 captures partial improvement of ≥50% in SLE disease activity in the most common disease manifestations in SLE. S2K RI-50 could be used as an outcome in clinical trials as a clinically meaningful measure of partial improvement.

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Enrique R. Soriano1, Hugo Javier Madariaga2, Oswaldo Castañeda3, Gustavo Citera4, Emilce E. Schneeberger4, Mario H. Cardiel5, Thijs Hendrikx6, Daniela Graham7, Harry Shi6, and Dario Ponce de Leon8. 1 Hospital Italiano de Buenos Aires, Buenos Aires, Argentina, 2 Clínica del Sur, Arequipa, Perú, 3 Clínica Angloamericana, Lima, Perú, 4 Instituto de Rehabilitación Psicofísica, Buenos Aires, Argentina, 5 Centro de Investigación Clínica de Morelia, Morelia, Mexico, 6 Pfizer Inc, Collegeville, USA, 7 Pfizer Inc, Groton, USA, 8Pfizer Inc, Lima, Perú.

Objectives: Non-alcoholic fatty liver disease, characterized by hepatic steatosis (HS), is a common form of chronic liver disease in many countries. Limited data are available on liver enzyme elevation in patients with HS receiving medications for inflammatory conditions such as rheumatoid arthritis (RA), psoriatic arthritis (PsA), and psoriasis (PsO). Tofacitinib is an oral Janus kinase inhibitor for the treatment of RA and PsA and has also been studied in PsO. The objective of this study was to describe baseline characteristics and liver enzyme abnormalities in patients from the tofacitinib RA, PsA, and PsO clinical programs with/without HS at baseline.

Methods: Patients randomized to the tofacitinib (5 or 10 mg twice daily; doses pooled) and placebo arms of 25 studies in the RA, PsA, and PsO clinical programs were included in this pooled post-hoc analysis. Most studies allowed or mandated concomitant treatment with disease-modifying antirheumatic drugs. HS was determined by the Investigator and captured per the Medical Dictionary for Regulatory Activities term at baseline. Baseline characteristics, incidence of elevated total bilirubin, aspartate aminotransferase (AST) and alanine aminotransferase (ALT) >1x and >3x the upper limit of normal (ULN) up to Month (M)3 and change from baseline in C-reactive protein (CRP) at M3 – all by HS at baseline – are reported.

Results: A total of 10,212 patients were included in the analysis. The prevalence of HS was 1.6% across indications (RA: 87/6,729 [1.3%]; PsA: 27/710 [3.8%]; PsO: 45/2,773 [1.6%]). Baseline characteristics were generally similar in patients with or without HS. However, baseline obesity, diabetes, triglycerides, and liver enzymes were numerically higher, and CRP was numerically lower, in patients with HS than in those without HS. In both tofacitinib- and placebo-treated patients, incidence of elevated total bilirubin, AST and ALT >1x ULN up to M3 was higher in patients with HS than in those without, across indications. Incidence of elevated total bilirubin, AST and ALT >3x ULN up to M3 was low across indications, irrespective of HS. Among tofacitinib-treated patients, CRP was reduced at M3 in patients with or without HS, but to a lesser extent in those with HS, across indications. Among placebo-treated patients, changes in CRP were small, irrespective of HS.

Conclusions: In this exploratory analysis, prevalence of HS at baseline was 1.6% across the tofacitinib RA, PsA, and PsO programs. After up to 3 months of tofacitinib treatment, incidence of mildly elevated liver enzymes was higher in patients with HS than in those without HS. Incidence of severely elevated liver enzymes was low overall, and similar in patients with or without HS. Further studies are needed to evaluate the effects of tofacitinib on CRP and liver enzymes – and the potential impact on clinical response – in patients with RA, PsA, or PsO who have comorbid HS.

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Florencia Pierini1, Eliana Botta2, Fernando Brites2, Laura Boero2, Maximiliano Martin2, Tomas Meroño2, Maria Soledad Saez2, Walter Teztlaff2, Fernando Sommerfleck3, Gustavo Citera3, Ignacio Gandino1, Javier Rosa1, Patricia Sorroche1, and Enrique R. Soriano1. 1 Hospital Italiano De Buenos Aires, Caba, Argentina, 2 Universidad de Buenos Aires, CABA, Argentina, 3Instituto de Rehabilitación Psicofísica, CABA, Argentina.

Objectives: The objective of this study was to compare the functional markers of HDL at baseline and 3 months after starting Tofacitinib in patients with RA.

Methods: Patients with RA (according to ACR / EULAR 2010 criteria) with de novo indication of tofacitinib were included as of January 2016. Patients with a personal history of cardiovascular disease, impaired renal, hepatic or thyroid function were excluded. DAS 28, lipid profile, and ultrasensitive C reactive protein were analyzed by standardized methods at baseline and 3 months after starting tofacitinib. PON1 activity was evaluated by two substrates, paraoxon (PON activity) and phenylacetate (ARE activity). Differences in the quantitative variables were analyzed with paired Wilcoxon test and qualitative variables with McNemar test. Spearman test was used for correlations.

Results: Rheumatoid factor was positive in 70.6% (CI 52.4-83.9) and anti-CCP was positive in 82.3% (CI 64.8-92.2). 18 patients (52.95%), had received biologics previously. Mean baseline DAS28 was 5.1 (SD 1.1). At three months, DAS28 decreased significantly (-24%, p <0.001). As expected, levels of TC, LDL-c, HDLc and Coleshterol-non-HDL (C-non-HDL) increased significantly after 3 months of treatment (TC: + 8%, p = 0.046, LDL-c: 8%, p = 0.046; HDL-c: + 8%, p = 0.027 and C-non-HDL: + 13%, p = 0.031). No changes were observed in PON activity or ARE activity associated with tofacitinib use (p> 0.05) in the total group. Patients without previous biologics had a significant increase in the activity of ARE and PON, while there were no changes in those who had failed a previous biologic No significant changes were observed in the CETP in any of the groups.

Conclusions: Despite an increase in lipoprotein levels, treatment with tofacitinib improved the antioxidant activity of HDL (paroxonase activity) in patients who had not received previous biologic. This could provide additional protection to the accelerated atherosclerotic process in those patients.

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Carolina Ayelen Isnardi1, Dafne Capelusnik1, Emilce Edith Schneeberger1, Marcela Bazzarelli2, Laura Barloco2, Eliana Soledad Blanco3, Cristian Alejano Benitez3, Federico Lujan Benavidez3, Santiago Scarafia4, María Alicia Lazaro4, Rodolfo Pérez Alamino5, Francisco Colombres5, Paula Kohan6, Julia Sosa6, Luciana Gonzalez Lucero7, Ana Lucía Barbaglia7, Hernan Maldonado Ficco8, and Gustavo Citera1. 1 Instituto De Rehabilitación Psicofísica, Caba, Argentina, 2 Hospital Interzonal General de Agudos Petrona V. de Cordero, Tigre, Argentina, 3 Hospital General de Agudos Dr. Cosme Argerich, CABA, Argentina, 4 Instituto de Asistencia Reumatológica Integral, San Isidro, Argentina, 5 Hospital de Clínicas Nicolás Avellaneda, San Miguel de Tucumán, Argentina, 6 Hospital General de Agudos Dr. Enrique Tornú, CABA, Argentina, 7 Hospital Ángel C. Padilla, San Miguel de Tucumán, Argentina, 8Hospital San Antonio de Padua, Rio Cuarto, Argentina.

Objectives: Depression is one of the most frequent comorbidities in Rheumatoid Arthritis (RA) patients. Its presence is associated with higher healthcare costs, mortality rate and reduced odds of achieving a good treatment response. The aim of this study was to determine the prevalence of depression in Argentinian patients with RA and to establish its relationship with different sociodemographic and clinical factors.

Methods: Consecutive patients ≥18 years of age, with a diagnosis of RA according to ACR-EULAR 2010 criteria were included. Sociodemographic data (sex, age, work and marital status, physical activity), comorbidities, RA characteristics (disease duration, RF and anti-CCP, presence of erosions), disease activity (joint count (28), composite indexes, pain, patient’s and physician´s global assessment-VAS-, morning stiffness), CRP, ESR and current treatment were registered. Questionnaires were administered to assess quality of life by EQ-5D-3L and QOL-RA, functional capacity by HAQ-A and depression by PHQ-9. Major depression was defined as a PHQ-9 score ≥10. PHQ-9 scores of 5-9, 10-14, 15-19, ≥20 represented mild, moderate, moderate/severe and severe depression, respectively. Patients with difficulties in answering the questionnaires (illiterate, blind) and with decompensated comorbidities were excluded. Statistical analysis: Student´s T, ANOVA and Chi2 tests. Multiple logistic regression.

Results: 258 patients were included. Median (m) disease duration of 9 years (IQR 3.6-16.7). DAS28-ESR was m 3.5 (IQR 2.5-4.5). Median PHQ-9 score was 6 (IQR 2-12.3). The prevalence of major depression was 33.8%. 66 (25.6%), 42 (16.3%), 27 (10.5%) and 18 (7%) patients presented mild, moderate, moderate/severe and severe depression, respectively. Patients with major depression had worst functional capacity (HAQ mean 1.56 ± 0.78 vs 0.69 ±0.65, p=0.0001), poorer quality of life (QOL-RA mean 5.38 ± 1.78 vs 7.31 ±1.61, p=0.0001), more pain (VAS mean 56.15 ± 27.48 mm vs 33.42 ± 25.72 mm, p=0.0001) and higher disease activity (DAS28-ESR mean 4.33 ±1.39 vs 3.3 ± 1.28, p=0.0001). Unemployment, presence of comorbidities, and less physical activity were more frequent in this group. In the multivariate analysis, worse functional capacity and quality of life were independently associated with the presence of major depression, independently of disease activity.

Conclusions: The prevalence of major depression measured by PHQ-9 score in this Argentinian cohort of rheumatoid arthritis patients was 33.8%. Depression had a significant impact on functional capacity and quality of life, regardless of disease activity.

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I. Mercedes-Núñez1, E. Tejada-Reyes1, M. Marte-Robles1, R. Jimenez-Candelaria1, V. Rosario1, T. Valdez-Lorie1, and R. Muñoz-Louis1, and R. Alba-Fériz1. 1Hospital Docente Padre Billini, Dominican Republic.

Objectives: To estimate the frequency of low serum vitamin D level (25(OH)D) in patients with rheumatoid arthritis (RA), and to analyze the association between 25(OH)D and disease activity.

Methods: A cross-sectional study. Data were collected from the rheumatology service of Hospital Docente Padre Billini, which is a national reference hospital. We included all patients older than 18 years diagnosed with RA according to the ACR / EULAR 2010 criteria, during the months of July-December 2017. Patients with low doses of 25(OH)D before the study period were excluded. 25(OH)D levels were classified as deficient <10 ng / mL, Insufficient 10-30 ng / mL, sufficient 30-100 ng / mL. Disease activity was measured using the disease activity score index (DAS28). Were analyzed using the statistical software SPSS V23.

Results: A total of 867 patients, 136 patients met inclusion criteria. The mean age was 58.4 years, 94.9 (127) were women, mean disease duration 8.6 years, the 25(OH)D levels were: 1.5% (2) deficient, 68.4% (93) insufficient, 30.1 % (42) sufficient. In relation to the activity levels, 51.4% (48) of the patients with low levels of 25 (OH) D had moderate-high activity disease. Sufficient levels of vitamin D had 50.4% (22) moderate-high activity.

Conclusions: There was high frequency of 25(OH)D deficiency (69.9%) in our cohort, but there was not a significant relationship between these levels and the activity of the disease in RA. We must measure the levels of vitamin D in the general population to compare with our RA cohort. Despite being a Caribbean island, we found a high frequency of low levels of vitamin D.

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Nathalie Alves1, Paola Marinheiro1, and Graziela Bernardino1. 1Bristol-Myers Squibb, Sao Paulo, Brazil.

Objectives: The Brazilian Society of Rheumatology recommends the use of various biological medications for the treatment of Rheumatoid Arthritis (RA), including abatacept (ABA) and adalimumab (ADA). As the number of treatment options increases for patients with RA, understanding treatment efficacy and how it may improve resource allocation are key elements of decision-making. This study aims to compare the cost-per-response for subcutaneous (SC) ABA and ADA for each clinical outcome available in the 2-year follow-up of the AMPLE study (ACR20, ACR50, ACR70, ACR90 and remission [DAS28 < 2.6]).

Methods: AMPLE was a non-inferiority study that compared the efficacy of subcutaneous (SC) ABA vs. ADA in adults with moderate to severe RA, diagnosed ≤ 5 years and after treatment failure with methotrexate monotherapy. Prices approved by Brazilian Regulation Chamber of Drug Market (CMED)* were considered for costs. The exchange rate used was 1USD = 3,79 BRL, average rate for November 2018. The costs for each therapy were calculated for 1 and 2 years of treatment (52 and 104 weeks, respectively). The cost-per-response was calculated for the following outcomes: ACR20, ACR50, ACR70, ACR90 and remission [DAS28 <2.6].

*The prices considered were the official listed prices approved for the Sao Paulo region, published on 11/19/2018 by CMED.

Results: In both periods, the cost-per-response ratio for all evaluated outcomes favors treatment with ABA SC compared to ADA. For one year of treatment, the cost-per-response in USD were: ACR20: 29,453.37 vs 43,170.50; ACR50: 41,311.22 vs 59,500.20; ACR70: 65,362.27 vs 104,466.00; ACR90: 183,517.15 vs 427,657.69; DAS28: 44,078.02 vs 65,322.42. For two years of treatment, the costs-per-response in USD were: ACR20: 63,938.97 vs 89,395.14; ACR50: 85,395.00 vs 115,292.87; ACR70: 122,738.16 vs 183,366.82; ACR90: 263,252.19 vs 655,200.95; DAS28: 75,437.88 vs 100,800.14.

Conclusions: The analysis, which was based on the clinical outcomes observed in the AMPLE study, showed that the costs-per-response for ABA SC were lower when compared to ADA in the two periods evaluated. The present study suggests that treatment with ABA SC can lead to a decrease in health care costs in the Brazilian private healthcare system compared to ADA, in first line biologic for MTX-inadequate responders, with similar results in terms of clinical efficacy.

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Ewa Mojs1, Michal Ziarko2, and Wlodzimierz Samborski3. 1 Poznan University of Medical Sciences, Department of Clinical Psychology, Poznan, Poland, 2 Adam Mickiewicz University, Institute of Psychology, Poznan, Poland, 3Poznan University of Medical Sciences, Department of Rheumatology and Rehabilitation, Poznan, Poland.

Objectives: Rheumatoid arthritis (RA) is a long-term disorder significantly impairing the somatic, emotional, and psychological functioning of its sufferers. Previous research has shown that affected individuals are characterized by an increased level of anxiety and depression. Currently, there are two main treatment schemes for RA; the first uses classic drugs modifying the activity of the disease, the second utilizes biologic agents modifying the activity of the disease. This begs the question whether sufferers differ in intensities of pain, anxiety, ad depression depending on the type of treatment, everyday functioning and what the determinants of these affective states in patients with RA.

Methods: The study comprised 150 patients affected by RA (including 70 receiving disease modifying biologic therapies). Research participants filled out a set of questionnaires measuring: levels of states of anxiety and depression, intensity of experienced pain, strategies of coping with pain, and ego resiliency as personality potence.

Results: The collected data was analyzed through intergroup comparisons, calculating simple correlation coefficients, developing and solving regression equations. The results imply that the choice of treatment differentiates the intensity of pain experienced by patients during the treatment and level of everyday functioning. Those receiving biologic agents reported lower levels of pain compared to those taking classic medication. It has also been noted that there are distinct configurations of conditions conducive to anxiety and depression in both groups. The occurrence of depression was much higher – appr 30% in comparison to healthy groups.

Conclusions: The observed constellation of dependencies between variables indicates that the choice of treatment scheme differentiates pain levels. This confirms the assumption that pain intensity, coping strategies, and ego resiliency depend on the severity of anxiety and depression.

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Gisele Luna1, Fernando Dal Pra1, Emilce Schneeberger1, and Macias Lysseth, Gustavo Citera1. 1Irep, Buenos Aires, Argentina.

Objectives: To evaluate the adherence to biological IV DMARDs (-b) in patients with RA in clinical practice and to determine the factors that could have an impact on the adherence.

Methods: An observational study was conducted involving patients ≥18 years of age, with a diagnosis of RA according to ACR / EULAR 2010 criteria, who received at least 3 infusions of the DMARD-b IV abatacept (ABA) or tocilizumab (TCZ), during the course of their illness. Medical records were reviewed to collect: sociodemographic data, clinical characteristics of the disease, comorbidities, disease activity (DAS28) and functional capacity (HAQ). Information was obtained on previous and concomitant treatments with b-DMARDs, including steroids and conventional DMARDs (-c), number of infusions of b-DMARD received, date of their initiation and discontinuation and causes of discontinuation. Adherence was calculated as the number of infusions received during the treatment period, considering a 28 days interval between infusions as optimal. Statistical analysis: descriptive statistics, Chi square test or Fisher's exact test, Student's T test and Mann Whitney test. Multiple logistic regression model to evaluate predictors of good adherence (≥80%).

Results: 67 patients included (94%women), median age of 55 years and a median disease duration 13.75years, 93.5% Rheumatoid Factor +, 85% had erosive disease. 82.1% had medical coverage and 60% corresponded to social work. Median distance to the infusion center was 15 km. Forty-two patients received ABA and 25 received TCZ. 72.4% received cs-DMARD concomitantly. The baseline DAS28 decreased from 5.46 to 3.8, p<0.001 at 3 months, and the baseline HAQ-A from 2 to 1, p<0.001. After a median follow-up time of 2.42 years, 38 patients (56.5%) discontinued definitively the IV b-DMARD. Causes of discontinuation were lack of efficacy (62.9%), AE (20%) and medication either not available or not covered (14.3%). Median survival was 4 years. Median number of infusions per year was 10.17(IQR 8.3-10.9), equivalent to an adherence of 78.2. Median infusions/year correlated negatively with distance to the infusion center (Rho:-0.27,p=0.003) and was significantly lower in patients without health coverage (8.5±2.5vs10.7±1.5,p<0.001). In multivariate analysis, having health coverage was the only variable associated with adherence ≥80% (OR:6.27,95%CI 1.1-37.9, p=0.04)

Conclusions: Adherence to treatment with IV b-DMARDwas acceptable, however our patients lose an average of 3 infusions per year. The only independent predictor associated with good adherence was having health coverage.

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Gustavo Citera1, Eduardo Mysler2, Hugo Madariaga3, Mario H Cardiel4, Oswaldo Castañeda5, Aryeh Fischer6, Pascal Richette7, Sandra Chartrand8, Jin Kyun Park9, Sander Strengholt10, Jose Luis Rivas11, Amit Thorat12, Tanya Girard13, Kenneth Kwok14, Lisy Wang15, and Dario Ponce de Leon16. 1 Instituto de Rehabilitación Psicofísica, Buenos Aires, Argentina, 2 Organización Médica de Investigación, Buenos Aires, Argentina, 3 Clínica del Sur, Arequipa, Perú, 4 Centro de Investigación Clínica de Morelia, Morelia, Mexico, 5 Clínica Anglo Americana, Lima, Perú, 6 Department of Medicine, University of Colorado Denver, Denver, USA, 7 Department of Rheumatology, Hôpital Lariboisière, Assistance Publique-Hôpitaux de Paris, Paris, France, 8 Department of Medicine, Hôpital Maisonneuve-Rosemont affiliated to Université de Montréal, Montréal, Canada, 9 Division of Rheumatology, Department of Internal Medicine, Seoul National University Hospital, Seoul, South Korea, 10 Pfizer Inc, Capelle aan den IJssel, Netherlands, 11 Pfizer SLU, Madrid, España, 12 Pfizer Ltd, Mumbai, India, 13 Pfizer Inc, Montréal, Canada, 14 Pfizer Inc, New York, USA, 15 Pfizer Inc, Groton, USA, 16Pfizer Inc, Lima, Perú.

Objectives: Tofacitinib is an oral Janus kinase inhibitor for the treatment of rheumatoid arthritis (RA). Interstitial lung disease (ILD) is a common extra-articular manifestation of RA.1 We investigated the incidence of ILD in patients with RA treated with tofacitinib in a post hoc analysis of data pooled from the tofacitinib clinical development program.

Methods: Incidence rates (IR; patients with events per 100 patient-years [PY]) of ILD events in patients with active RA, receiving tofacitinib 5 or 10 mg twice daily, were calculated using data pooled from 2 Phase (P)1, 10 P2, 6 P3, 1 P3b/4, and 2 long-term extension (LTE) trials (ORAL Sequel LTE main study database locked at time of analysis: March 2017). No patients with pre-existing ILD were included. Potential ILD events were adjudicated by 3 independent pulmonologists as ‘probable’ (compatible adverse event [AE] with supportive clinical evidence) or ‘possible’ (compatible AE with no supportive clinical evidence). ILD IRs are described by patient age and region, and over time. In P2/3/3b/4 studies, ILD IRs were compared for tofacitinib vs placebo. A descriptive case-matched control (1:5 ratio of patients with vs without ILD events, matched by age and gender) analysis identified potential ILD risk factors.

Results: Of 7061 patients (23,394 PY exposure), 42 (0.6%) had an ILD event (median time to event 1144 days). The ILD IR was 0.18 with both tofacitinib doses. IRs were numerically higher in patients aged ≥65 vs <65 years and Asian vs non-Asian countries; 95% confidence intervals were wide/overlapping. IRs generally remained stable over time. 17/42 ILD events (40.5%) were serious; 35/42 (83.3%) were mild to moderate in severity. ILD IRs were numerically lower with tofacitinib vs placebo. A numerically higher proportion of patients in the ILD group (n=42) vs control group (n=210) were: Asian (31.0% vs 17.6%); smokers/ex-smokers (50.0% vs 39.5%); RF-positive (89.2% vs 71.0%); anti-CCP antibody positive (54.8% vs 46.7%); had received prior MTX (90.5% vs 79.5%), non-MTX csDMARDs (61.9% vs 55.2%), TNF inhibitors (26.2% vs 18.6%), and concomitant glucocorticoids (71.4% vs 52.9%); and had higher baseline mean ESR (57.0 vs 46.9 mm/hr) and CRP (25.4 vs 15.4 mg/L).

Conclusions: In this post hoc analysis of data from P1/2/3/3b/4/LTE studies, ILD events following tofacitinib treatment were low, and were associated with known risk factors.


1. Curtis J et al. Arthritis Res Ther 2015; 17: 319.

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María Lorena Brance1,2, Bernardo A Pons-Estel3, Norberto Javier Quagliato4, Marisa Jorfen5, Guillermo Berbotto6, Noel Cortese5, Juan C Raggio6, Juan Soldano4, Mariano Palatnik7, Ignacio Chavero1, Carolina Dieguez1, Ariel Sánchez8, Luis del Rio9, Silvana Di Gregorio9, and Lucas R. Brun2,10. 1 Reumatología y Enfermedades Óseas, Rosario, Argentina, 2 Lab. Biología Ósea. Univ. Nacional de Rosario., Rosario, Argentina, 3 Centro Regional de Enfermedades Autoinmunes y Reumáticas (CREAR), Rosario, Argentina, 4 Hospital Provincial, Rosario, Argentina, 5 Red Municipal, Rosario, Argentina, 6 Hospital Eva Perón, Baigorria, Argentina, 7 Centro de Reumatología, Rosario, Argentina, 8 Centro de Endocrinología., Rosario, Argentina, 9 CETIR, Barcelona, España, 10Consejo Nacional de Investigaciones Científicas y Técnicas (CONICET), Argentina.

Objectives: Rheumatoid arthritis (RA) is a chronic autoimmune disease characterized by symmetric polyarthritis that can lead to joint deformity and disability. In addition, bones and muscles are also affected. Local osteoporosis develops first and then, it can progress to generalized osteoporosis. Sarcopenia is characterized by a progressive and generalized loss of muscle mass and strength. Aim: To evaluate the muscle mass and the prevalence of sarcopenia in patients with RA.

Methods: Adult RA patients (n=105) of both sexes and 100 subjects as control group (CG) matched by age, sex and body mass index (BMI) were included. Exclusion criteria: pregnancy, intestinal malabsorption, chronic liver or kidney disease, cancer and drug that could affect bone mass except glucocorticoids. Whole-body composition was measured by dual-energy X-ray absorptiometry (DXA, Hologic Discovery Wi). Sarcopenia was defined as low muscle mass (appendicular skeletal muscle mass/height2 and low muscle function: muscle strength and/or physical performance). Muscle strength was evaluated by hand-grip strength (Baseline Hand Dynamometer, USA) in the dominant hand. The physical performance was evaluated by sit to stand, timed up and go and 8-foot walking test. The distribution of the data was evaluated with the Shapiro-Wilk test and the differences between groups were analyzed using the Student's t test or the Mann-Whitney test, as appropriate. Data were expressed as mean±SD and p<0.05 was considered significant.

Results: No differences in age, sex (CG: 84/16 F/M, RA: 87/18 F/M) and BMI were found between groups. Lower percentage of lean mass was found in RA patients (CG: 59.5%, RA: 57.0%; p=0.0120). In addition, lower muscle strength and/or physical performance in RA were found (handgrip strength= CG: 26.4±8.3 kg, RA: 17.7±8.9 kg, p<0.0001; sit to stand test= CG: 12.8±5.9 s, RA: 16.5±5.9, p<0.0001; timed up and go test= CG: 7.5±2.0 s, RA: 10.3±3.5 s, p<0.0001; 8-foot walking test= CG: 1.07±0.27 m/s, RA: 0.79±0.23 m/s, p<0.0001). Therefore, higher prevalence of sarcopenia in RA group was found (CG: 8.1%; RA: 44.3%; RR: 2.1; p<0.0001). While in the CG there was no cases of severe sarcopenia, in the RA group 33% were found. Moreover, a higher percentage of total fat (GC: 37.3±7.2%, RA: 40.9±6.8%, p=0.0062) and visceral fat (GC: 566.2±305.8 g, RA: 692.7±350.0 g, p=0.0090) were found in patients with RA.

Conclusions: A higher prevalence of sarcopenia was found in RA patients with an increase in total and visceral fat. The activity of the disease, immobility and treatment with glucocorticoids would affect the muscle mass in patients with RA