Low Rates of Reproductive Counseling Documentation in Women With Interstitial Pneumonia With Autoimmune Features : JCR: Journal of Clinical Rheumatology

Secondary Logo

Journal Logo

Original Article

Low Rates of Reproductive Counseling Documentation in Women With Interstitial Pneumonia With Autoimmune Features

Joerns, Elena K. MD; Mills, Brooke MD; Makris, Una E. MD, MSc∗,†; Adams, Traci N. MD; Bermas, Bonnie MD

Author Information
JCR: Journal of Clinical Rheumatology ():10.1097/RHU.0000000000001929, November 24, 2022. | DOI: 10.1097/RHU.0000000000001929
  • Open
  • PAP


Interstitial lung disease (ILD) has various presentations including rheumatic disease (RD) associated ILD and idiopathic pneumonias. Some patients with ILD do not clearly fall into either of these categories yet have features of autoimmunity. In 2015, European Respiratory Society and American Thoracic Society issued a joint statement for the classification of such patients and coined the term interstitial pneumonia with autoimmune features (IPAF).1 Similar to RD-ILD, IPAF affects women more frequently than men and the age of onset tends to be younger than some other ILD subtypes such as idiopathic pulmonary fibrosis.2,3

In 2020, the American College of Rheumatology issued Guidelines on the Management of Reproductive Health in Rheumatic and Musculoskeletal Diseases.4 The guidelines emphasized the need for family planning discussions in RD patients. Although these guidelines help practitioners navigate family planning in women with established RDs, they do not explicitly extend to women with ILD without a defined RD. Thus, there is little guidance on reproductive health counseling in IPAF patients despite the finding that family planning discussions improve pregnancy outcomes in patients with ILD.5,6

Pregnancy outcomes among ILD patients are variable.6,7 Some ILD patients have healthy pregnancies with minimal complications under close observation.6 However, pregnancy in women with uncontrolled pulmonary disease is high risk and has been associated with intrauterine growth restriction and maternal respiratory failure during labor and delivery.5 Few studies have focused on outcomes for pregnancies of RD-ILD patients, and there are no studies that have focused on pregnancy outcomes for IPAF patients specifically.6–8 Moreover, IPAF is frequently complicated by pulmonary vascular disease, including pulmonary hypertension (PH).9 Pulmonary hypertension is associated with high rates of maternal mortality,10 and current guidelines recommend against pregnancy in this patient population.11,12 In addition, SSA and SSB antibodies are frequently encountered in IPAF patients2 and are associated with neonatal lupus and congenital complete heart block.4

The mainstay of therapy for IPAF consists of potentially teratogenic immunomodulating medications.13 Thus, reproductive-aged female patients with IPAF need to be counseled about the importance of highly effective contraception while taking these medications. Pregnancy planning not only protects against inadvertent exposure to teratogenic medications but also ensures that patients have well-controlled disease on pregnancy compatible medications before conception.

The objective of this study was to evaluate the frequency of reproductive counseling documentation including family planning discussions and documentation of contraceptive use by rheumatology and pulmonary providers with female reproductive-aged IPAF patients seen at an academic medical center.


Cohort Assembly

This single-center medical record review study was conducted at UT Southwestern Medical Center (UTSW). Consecutive patients seen in the UTSW ILD Clinic between January 2005 and April 2019 who met the ERS/ATS classification criteria for IPAF1 were identified, using a preexisting database established by coauthor (T.N.A.). For patients seen before 2015, IPAF classification criteria were retrospectively applied. Pulmonologist (T.N.A.) initially assigned patients to IPAF classification with rheumatologist (E.K.J.) subsequently and independently verifying the classification. Female patients aged 18 to 50 years at the time of initial ILD clinic visit were included. The UTSW Medical Center Institutional Review Board approved the study before initiation of data extraction (IRB #STU-2019-0913). Patient consent was not required by the institutional review board for this medical record review study.

Data Collection and Variables

All data were extracted from the electronic medical record (EMR) by a rheumatologist (E.K.J.).

Female patients aged 18 to 50 years at the initial pulmonary ILD clinic visit and who met classification criteria for IPAF were eligible for inclusion in this study. Data extraction tools for consistent data collection and data recording in the worksheet were generated by rheumatologists E.K.J. and B.M. for this study. If data were missing (ie, SSA antibody), it was considered that the patient was not positive for the presence of that data variable.

We collected data from pulmonary and rheumatology clinic visits, medical, and surgical history as documented in EMR, medication lists, and procedure records. We extracted data on demographics, pulmonary function tests, PH data, medication use, SSA and SSB positivity, and documentation of family planning counseling and/or contraceptive use by pulmonary and rheumatology providers, during any visit, over the duration of the follow-up at ILD clinic. We also extracted data on whether the patient was seen by obstetrics/gynecology (OB/GYN) providers during their follow-up at ILD clinic. Both pulmonology and rheumatology notes were generated by faculty, advanced practice providers, and trainees (clinical fellows). The UTSW uses the Epic EMR system, which is shared among all divisions at UTSW. All but one of the patients seen at the rheumatology clinic were also seen at the ILD clinic concurrently. One patient was seen by a rheumatology provider after her last visit with pulmonary provider.


Demographic data included age at ILD diagnosis, age at first and last ILD clinic visit, duration of follow-up at ILD clinic, sex, and race/ethnicity as documented in EMR. ILD diagnosis date was the date at which ILD was first observed on imaging. Race and ethnicity were documented in the EMR using prespecified categories (White; Black or African American; Asian, American Indian or Alaska Native, Native Hawaiian or other Pacific Islander, unavailable/unknown, other race, or declined; and Hispanic or Latino, non-Hispanic/Latino, declined, or unknown) and not confirmed with the patient.

Pulmonary Function Test Data

Pulmonary function test data included forced vital capacity (FVC) and diffusing lung capacity for carbon monoxide (DLCO), expressed as percentages of predicted values, at initial ILD clinic visit.

Pulmonary Hypertension Data

Pulmonary hypertension data included right heart catheterization (RHC) parameters with mean pulmonary artery pressure, pulmonary capillary wedge pressure, and pulmonary vascular resistance. Precapillary PH presence was defined by RHC parameters with mean pulmonary artery pressure over 20 and pulmonary capillary wedge pressure ≤15 mm Hg.14 All patients carrying a diagnosis of PH had an RHC performed at UTSW.

Medication Use

Data on immunomodulatory therapy (prednisone, azathioprine, mycophenolate mofetil, cyclophosphamide, hydroxychloroquine, and rituximab) including types of medications and dates of treatment were extracted from each visit encounter medication list for the duration of the follow-up period.

Reproductive Counseling Documentation

Reproductive counseling documentation was assessed by presence of documentation of family planning counseling (such as recommendation for contraception or referral to OB/GYN provider) and/or contraceptive use by pulmonary and rheumatology providers, during any visit. Documentation had to be present in the visit note by the provider. Autopopulation of data was included as evidence of documentation as the provider had to manually choose to import the data in the note for it to autopopulate. Prior sterilization such as tubal ligation or hysterectomy was included as a contraception method if it was documented in any visit note.

Primary Outcome

The primary outcome was proportion of patients with reproductive counseling documentation, defined as documentation of either family planning counseling or ongoing contraceptive use (including prior sterilization) by pulmonary and rheumatology providers during any visit.

Statistical Analysis

Descriptive statistics were used to describe data in EMR and progress notes from pulmonary and rheumatology providers (physicians including trainees and advanced practice providers).

Categorical variables were expressed as counts with percentages, and continuous variables were expressed as mean with standard deviation (SD). Missing data were not analyzed; sample sizes are listed where appropriate. Nonparametric measures of association (χ2, Fisher exact, Mann-Whitney U test) were used, as appropriate, to evaluate the relationship between baseline characteristics and reproductive counseling documentation by providers. We performed logistic regression to evaluate association of baseline demographic variables with odds of reproductive counseling documentation. Statistical Software Stata V.1715 was used for analysis.

Data Availability Statement

The data sets generated and analyzed during the current study are available from the corresponding author on reasonable request.



Two hundred one patients with IPAF were screened for eligibility for inclusion in this study. Forty-nine patients were excluded for being male. Of the remaining 152 patients, 31 women were aged 50 years or younger at initial ILD clinic visit and thus were included in the study (Fig. 1).

Participant inclusion in the study.

Baseline Characteristics

Baseline characteristics of female patients with IPAF followed at UTSW ILD clinic and who were included in the study are described in Table 1. A total of 31 women met IPAF classification and were ≥18 years and ≤50 years (mean age of 43.2 ± 5.9 years) at initial ILD clinic visit. The mean duration of follow-up at ILD clinic was 4.4 ± 3.7 years. Fifteen women identified as White, 11 as Black or African American, 1 as Asian, 1 as American Indian/Alaska Native, and 3 as Other Race. Seven women (23%) identified as Hispanic/Latino. The mean baseline FVC was 58.2% ± 19.5%, and baseline DLCO was 38.1% ± 25.1%. Twenty-five women (81%) had risk factors for adverse pregnancy outcomes, including positive SSA/SSB antibodies, presence of PH, exposure to teratogenic medications, and/or reduced %FVC (Table 1). Sixteen women (52%) had positive SSA antibody (1 had concurrent positive SSB antibody), and 12 women (39%) had PH by RHC parameters.14 Thirty (97%) women were on immunomodulatory therapy (hydroxychloroquine, azathioprine, mycophenolate mofetil or mycophenolic acid, rituximab, intravenous immunoglobulin, cyclophosphamide, prednisone, etanercept) at some point during follow-up, with 25 (81%) women taking teratogenic medications (mycophenolate mofetil and cyclophosphamide). Twenty-three patients were seen by OB/GYN providers during follow-up; of the 8 patients not seen by OB/GYN, only 2 had a documentation of prior sterilization (tubal ligation and hysterectomy). Of the 23 patients followed by OB/GYN providers concurrently, only 3 patients had documentation of this. Fourteen patients (45%) had a hysterectomy or tubal ligation before or during the follow-up period. Menopause was not documented for any of the women.

TABLE 1 - Baseline Characteristics of Female Patients With IPAF Followed at ILD Clinic
Baseline Characteristics Female Patients With IPAF (n = 31)
Patients seen in rheumatology clinic 21 (68)
Documentation of family planning counseling or contraceptive use in visit note, n (%) 10 (32)
Mean age at dx (SD), y 41.4 (5.9)
Mean age at first ILD clinic visit (SD), y 43.2 (5.9)
Race, n (%)
 White 15 (48)
 Black 11 (35)
 Asian 1 (3)
American Indian 1 (3)
 Other 3 (10)
Hispanic ethnicity, n (%) 7 (23)
Teratogenic medications,a n (%) 25 (81)
SSA positive, n (%) 16 (52)
%FVC (SD) 58.2 (19.5)
%DLCO (SD) 38.1 (25.1)
Presence of PH, n (%) 12 (39)
Sterilization history,b n (%) 14 (45)
Seen by OB/GYN providers, n (%) 23 (74)
aTherapy with potentially teratogenic medications during follow-up at ILD clinic.
bTubal ligation and/or hysterectomy.
SSA, anti–Sjögren syndrome–related antigen A autoantibody.

Documentation of Reproductive Counseling by Pulmonary Providers

Ten of 31 women with IPAF (32%) who saw pulmonary providers had documentation of contraception or family planning discussion in any note (Fig. 2). Four women had family planning discussion with their pulmonary providers, and 6 women had a documentation of contraception use in the note. Only 3 women (10%) with no record of contraception use in the note had a documented family planning discussion.

Reproductive counseling documentation frequency by pulmonary and rheumatology providers in clinic encounters with female patients with IPAF of childbearing age.

Documentation of Reproductive Counseling by Rheumatology Providers

Of 21 patients with IPAF who saw rheumatology providers at UTSW, 12 of 21 (57%) had documented family planning counseling or contraception by rheumatologists. Two had family planning discussion documented in the encounter note, and additional 10 patients had documentation of active contraceptive use in the visit note (Fig. 2). There was a nonstatistically significant trend for rheumatology providers to be more likely to document family planning discussion and/or contraception than pulmonary providers (Table 2).

TABLE 2 - Baseline Characteristics of Women With IPAF With Reproductive Counseling Documentation
Characteristics Pulmonary Providers (n = 10) Rheumatology Providers (n = 12a) p value
Mean age at diagnosis (SD), y 42.5 (6.9) 39.9 (5.5) 0.31
Mean age at first ILD clinic visit (SD), y 42.8 (6.9) 40.1 (5.5) 0.25
Race, n (%) 0.41
 White 4 (40) 4 (19)
 Black 5 (50) 6 (29)
 Asian 0 (0) 1 (5)
American Indian 1 (10) 0 (0)
 Other 0 (0) 1 (5)
Hispanic, n (%) 1 (10) 1 (8) 1
Teratogenic medications, n (%) 9 (90) 10 (83) 1
SSA positive, n (%) 8 (80) 7 (58) 0.38
Mean %FVC (SD) 59.5 (15.8) 63.7 (23.7) 0.69
Mean %DLCO (SD) (n = 19) 37.4 (17.0) (n = 9) 44.2 (27.5) (n = 10) 0.73
PH present, n (%) 4 (40) 4 (33) 1
Sterilization history,b n (%) 7 (70) 6 (50) 0.41
Seen by OB/GYN providers, n (%) 8 (80) 12 (100) 0.19
aPatients also seen in the ILD clinic.
bTubal ligation and/or hysterectomy.
SSA, anti–Sjögren syndrome–related antigen A autoantibody.

Counseling of Women With Additional Risk Factors

Of the 25 women on teratogenic medication seen by pulmonary providers, 9 women (36%) had documentation of either ongoing contraceptive use or discussion of family planning in the visit note, in contrast to 10 of 17 women (59%) on teratogenic medications seen by rheumatology providers. This trend was not statistically significant (Table 2). Women who were SSA positive had somewhat higher odds of having reproductive counseling documentation (odds ratio, 6.5; 95% confidence interval, 1.09–38.63; p = 0.040); none of the other characteristics were significantly predictive of reproductive counseling documentation (Table 3).

TABLE 3 - Effect of Baseline Characteristics on Odds of Reproductive Counseling Documentation in Women of Childbearing Age With IPAF by Pulmonary and Rheumatology Providers in Unadjusted Analysis
Pulmonary Providers (n = 31) Rheumatology Providers (n = 21a)
Baseline Characteristic OR (95% CI) p value OR (95% CI) p value
Black race 2.29 (0.44–11.92) 0.324 3.75 (0.47–29.75) 0.211
Hispanic ethnicity 0.28 (0.029–2.70) 0.269 0.18 (0.015–2.15) 0.177
Age at diagnosis 1.05 (0.92–1.20) 0.459 0.94 (0.81–1.09) 0.437
Age at first ILD clinic visit, (SD), y 0.98 (0.86–1.12) 0.809 0.88 (0.75–1.04) 0.132
Teratogenic medications 2.81 (0.28–27.97) 0.378 1.43 (0.16–12.70) 0.749
SSA positivity 6.5 (1.09–38.63) 0.040 1.12 (0.20–6.41) 0.899
Baseline %FVC 1.01 (0.97–1.05) 0.798 1.04 (0.99–1.11) 0.130
Baseline %DLCO 1.00 (0.97–1.03) 0.919 1.05 (0.99–1.11) 0.134
PH presence 1.00 (0.20–5.00) 1 0.43 (0.062–2.97) 0.391
Sterilization historyb 4.67 (0.92–23.79) 0.064 1.25 (0.22–7.08) 0.801
Seen by OB/GYN providers 1.6 (0.26–9.83) 0.612 Omitted due to collinearity NA
aPatients also seen in the ILD clinic.
bTubal ligation and/or hysterectomy.
OR, odds ratio; CI, confidence interval; SSA, anti–Sjögren syndrome–related antigen A autoantibody.

Counseling of Women Based on Demographic Factors

Differences in the rates of counseling by pulmonary and rheumatology providers based on baseline characteristics of women are described in Table 2. None of the demographic characteristics were significantly predictive of reproductive counseling documentation (Table 3).

Similar proportions of Black and White women had reproductive counseling documented during both pulmonary and rheumatology notes, although numerically fewer of the Hispanic women (1 of 7 who self-identified as Hispanic or Latino) had either contraceptive use or family planning discussion documented during any of their visits with either specialty. No other baseline characteristics contributed to documentation rates of reproductive counseling by rheumatology providers.


In our study, family planning discussions and/or contraceptive counseling documentation among reproductive-aged women with IPAF was found in 33% of pulmonary encounters and 57% of rheumatology encounters. Although rheumatology providers were more likely to document discussions of family planning with their patients or ongoing contraception use than their pulmonary colleagues, this trend was not statistically significant. Use of teratogenic medications in these patients did not impact the rates of documentation. There was a nonsignificant trend for Hispanic patients to have lower percentage of reproductive counseling documentation. Patient's status of being seen by OB/GYN provider did not affect the odds of documentation of family planning discussion and contraception use by pulmonary and rheumatology providers.

Other studies have corroborated our findings of low rates of reproductive counseling between providers and high-risk patients. In a study of PH providers, only 9% of physicians reported discussing reproductive issues with patients at every visit.10 Black and Hispanic/Latina women had lower odds of receiving contraceptive counseling,16 which was consistent with our study, with fewer Hispanic women having a documentation of reproductive counseling or contraceptive use. Similarly, the rheumatology literature also reports inconsistent family planning discussions with patients who would have high-risk pregnancies. Women with systemic lupus erythematosus (SLE) on teratogenic medications were no more likely to receive contraceptive counseling than women on nonteratogenic medications.17

Several barriers could explain the inconsistent reproductive counseling documentation among the providers in our study. Insufficient encounter time, lack of ownership of this health care issue, lack of provider knowledge regarding family planning, and provider discomfort and/or patient resistance in discussing sensitive topics such as contraception and family planning could all contribute.10 In addition, IPAF patients are complicated, and numerous topics may need to be discussed each visit, limiting the time for addressing health care maintenance such as reproductive health.

Strategies to improve both the presence and documentation of reproductive counseling among these at-risk patients include previsit questionnaires and nurse-led protocols. Education of both pulmonary and rheumatology providers on the importance of this topic could potentially increase these discussions. Other studies have shown that among rheumatology providers, simple interventions such as open-ended questions by providers regarding plans for pregnancy in the next year increase the family planning counseling in the clinic.18 In a recent study, women with RDs strongly wished for their rheumatology providers to initiate the discussion of pregnancy planning, highlighting the need for providers to initiate patient-provider communication on this topic.19

Our study has several limitations including having a small sample size and being medical record review study, which may have led to missing data. It is possible that we did not capture all women with IPAF of childbearing age because we limited our cohort to patients seen in UTSW ILD clinic. Because all but 1 patient seen by rheumatology providers were also concurrently seen in pulmonary clinic, it is possible that, based on timing of visits and documentation, counseling was influenced by reviewing the other clinics' notes. Furthermore, we only evaluated documentation of counseling and birth control use based on the visit notes, which excludes nondocumented reproductive counseling.

Our study has several strengths. To our knowledge, this is the only study evaluating reproductive counseling documentation in IPAF patients specifically, and our findings highlight the need to address this health care gap in this particular population. We have access to both pulmonary and rheumatology clinic notes within the same medical center, which improves internal validity of the study and eliminates intercenter and interregional variability in the approach to family planning counseling.

In summary, this study highlights a critical health maintenance gap in the care of female patients with IPAF. Future studies will focus on elucidating reasons for the lack of counseling and counseling documentation, with the ultimate goal of improving awareness to counsel, strategies to communicate the risks, and mitigation measures to optimize pregnancy outcomes in this high-risk patient population.


  • Women with IPAF have risk factors for adverse pregnancy outcomes.
  • Women with IPAF have a health care gap.
  • Providers do not document reproductive counseling consistently.
  • No baseline characteristic was associated with counseling documentation in women with IPAF.


Dr Elena K. Joerns was supported by T32 Ruth L. Kirschstein Institutional National Research Service Award (grant no. T32HL098040-1, PI: Lance Terada) for conduct of this research.


1. Fischer A, Antoniou KM, Brown KK, et al. An official European Respiratory Society/American Thoracic Society research statement: interstitial pneumonia with autoimmune features. Eur Respir J. 2015;46:976–987.
2. Oldham JM, Adegunsoye A, Valenzi E, et al. Characterisation of patients with interstitial pneumonia with autoimmune features. Eur Respir J. 2016;47:1767–1775.
3. Chartrand S, Swigris JJ, Stanchev L, et al. Clinical features and natural history of interstitial pneumonia with autoimmune features: a single center experience. Respir Med. 2016;119:150–154.
4. Sammaritano LR, Bermas BL, Chakravarty EE, et al. 2020 American College of Rheumatology Guideline for the Management of Reproductive Health in Rheumatic and Musculoskeletal Diseases. Arthritis Rheumatol. 2020;72:529–556.
5. Mylvaganam R, Dua S, Nelson-Piercy C, et al. Interstitial lung disease in women of child-bearing age. Semin Respir Crit Care Med. 2017;38:185–190.
6. Clowse MEB, Rajendran A, Eudy A, et al. Pregnancy outcomes in patients with interstitial lung disease. Arthritis Care Res (Hoboken). 2021.
7. Boggess KA, Easterling TR, Raghu G. Management and outcome of pregnant women with interstitial and restrictive lung disease. Am J Obstet Gynecol. 1995;173:1007–1014.
8. Lapinsky SE, Tram C, Mehta S, et al. Restrictive lung disease in pregnancy. Chest. 2014;145:394–398.
9. Chung JH, Montner SM, Adegunsoye A, et al. CT findings, radiologic-pathologic correlation, and imaging predictors of survival for patients with interstitial pneumonia with autoimmune features. AJR Am J Roentgenol. 2017;208:1229–1236.
10. Hill W, Holy R, Traiger G. EXPRESS: intimacy, contraception, and pregnancy prevention in patients with pulmonary arterial hypertension: are we counseling our patients?Pulm Circ. 2020;10:1–10.
11. Hemnes AR, Kiely DG, Cockrill BA, et al. Statement on pregnancy in pulmonary hypertension from the pulmonary vascular research institute. Pulm Circ. 2015;5:435–465.
12. Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS guidelines for the diagnosis and treatment of pulmonary hypertension: the Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Respir J. 2015;46:903–975.
13. Joerns EK, Adams TN, Newton CA, et al. Variables associated with response to therapy in patients with interstitial pneumonia with autoimmune features. J Clin Rheumatol. 2022;28:84–88.
14. Kovacs G, Olschewski H. The definition of pulmonary hypertension: history, practical implications and current controversies. Breathe (Sheff). 2021;17:210076.
15. StataCorp. Stata Statistical Software: Release 17. College Station, TX: StataCorp LLC; 2021.
16. Agénor M, Pérez AE, Wilhoit A, et al. Contraceptive care disparities among sexual orientation identity and racial/ethnic subgroups of U.S. women: a National Probability Sample Study. J Womens Health (Larchmt). 2021;30:1406–1415.
17. Yazdany J, Trupin L, Kaiser R, et al. Contraceptive counseling and use among women with systemic lupus erythematosus: a gap in health care quality?Arthritis Care Res (Hoboken). 2011;63:358–365.
18. Birru Talabi M, Clowse MEB, Schwarz EB, et al. Family planning counseling for women with rheumatic diseases. Arthritis Care Res (Hoboken). 2018;70:169–174.
19. Wolgemuth T, Stransky OM, Chodoff A, et al. Exploring the preferences of women regarding sexual and reproductive health care in the context of rheumatology: a qualitative study. Arthritis Care Res (Hoboken). 2021;73:1194–1200.

reproductive counseling; women's health; interstitial pneumonia with autoimmune features; risk factors for adverse pregnancy outcomes; interstitial lung disease

Copyright © 2022 The Author(s). Published by Wolters Kluwer Health, Inc.