JCR: Journal of Clinical Rheumatology:
Efe, Cumali MD*; Urün, Yuksel MD*; Purnak, Tugrul MD†; Ozaslan, Ersan MD†; Ozbalkan, Zeynep MD‡; Savaşs, Berna MD§
From the *Internal Medicine Department, Ankara Numune Research And Education Hospital, Ankara, Turkey; and †Gastroenterology Department, ‡Rheumatology Department, and §Pathology Department, Ankara University School of Medicine, Ankara, Turkey.
Correspondence: Cumali Efe, MD, Yazgan Sokak 21/12 Cebeci Ankara, Turkey. E-mail: firstname.lastname@example.org.
Celiac disease (CD) is an autoimmune disorder that occurs in genetically predisposed individuals as the result of gluten ingestion. The estimated prevalence of CD is approximately 1% to 2% in Europe and the United States but the disease pattern and prevalence seem to be changing in recent years. Although CD can start at any age, it presents most commonly in either early childhood or in the third or fourth decade of life. It can also be diagnosed in patients older than 60 years. CD is divided in 2 clinical subtypes; the classic or symptomatic type presents with the following gastrointestinal symptoms: abdominal discomfort, pain, distension, diarrhea, and weight loss. In atypical or occult forms the extraintestinal features dominate like iron deficiency anemia, arthritis, osteoporosis, constipation, and infertility.1,2 Articular involvement has been reported prevalently in CD but rarely in arthritis as first clinical presentation before the gastrointestinal symptoms. We report here the clinical case of a patient whose presenting symptom was symmetric polyarthritis before the diagnosis of CD.
A 34-year-old woman presented with 2-month history of malaise, polyarthralgia, and joint swelling. There was neither significant history of altered bowel habit nor significant medical history. Her family history was negative for rheumatic and inflammatory bowel diseases. Corticosteroid and nonsteroidal anti-inflammatory drug treatment were prescribed. She had taken these irregularly for the last 2 months, but her symptoms never resolved completely. When she was admitted to our clinic, she looked ill; and her initial physical examination revealed 37.6°C temperature, 88/min heart rate, and 120/70 mm Hg blood pressure. Joint examination revealed warmth and moderate soft tissue swelling, tenderness, and pain on motion in bilateral on knees, wrists, proximal interphalangeal, and metacarpophalangeal joints. Her anterior flexion of lumbar spine (Schober test) +3 cm and sacroiliac compression tests were positive. Initial laboratory values were as the follows: hemoglobin 10.5 g/dL, white blood cell count 6700/mm3, mean corpuscular volume 69 fL (normal: 80–97), platelets 278000/mm3, blood iron 17 μg/dL (normal: 30–80), total iron binding capacity 450 μg/dL (normal: 250–425), and ferritin level 5 ng/mL (normal: 28–365). Liver function tests, urea, creatinine electrolytes, LDH albumin, and total protein were normal. Immune globulins, C3, C4, rheumatoid factor, anti nuclear antibody, tests for marker of hepatitis B and C virus were negative. HLA B27, HLA B8, and DR3 were positive. CRP level was 47 mg/L (normal: 0–5) and ESR was 125 mm/h. X-ray studies of sacroiliac joints were not specifically classified as sacroiliitis but computed tomography scanning showed abnormal sacroiliac joints with erosions and pseudowidening. Also Tc99 pertechnetate bone scanning showed increased inflammatory activity in both sacroiliac joints. Upper and lower intestinal endoscopies were performed to exclude inflammatory bowel diseases and any malabsorbtive disorders. Lower intestinal endoscopy was normal but upper intestinal endoscopy showed loss of duodenal folds and mosaic pattern. With these findings, biopsy was taken and serological tests were ordered. Serological markers for CD revealed that antigliadin and antitissue transglutaminase antibodies were >200 Ru/mL (normal range, 0–50 Ru/mL). Antiendomysium was measured quantitatively and it was (+4), which refers to strong positivity. Duodenal biopsy showed flat duodenum mucosa due to villous atrophy with crypt elongation and increased intraepithelial lymphocytes (Figure 1). A gluten-free diet was started upon the diagnosis of CD. Her joint symptoms resolved in a 4 weeks. All laboratory abnormalities returned to normal levels after 1-year follow-up.
Classic CD is recognized easily but, however, atypical or silent types of patients are diagnosed incidentally or during the searching for the causes of anemia, osteoporosis, arthritis, or unexplained infertility.1,2 Several studies showed that there is significant delay of time between the initial appearance of symptoms and the diagnosis of CD.2,3 Until the last decade, CD was considered to be a rare condition, but now it is known as one of the most common disease with a mean prevalence of 1%to 2% in the general population.1,3 The natural history and presentation of CD seems to be changing. Diarrhea has become a less common presentation than it once was. Many patients, especially adults, have minimal symptoms, and gastrointestinal symptoms are frequently absent. Articular involvement is reported in patients with CD and usually presents as acute nonerosive arthritis, involving axial or peripheral joints. Adelizzi et al reported a patient with CD who presented with seronegative oligoarthritis, Bourne et al reported 6 patients, Slot et al reported 2 cases whose arthritis was prominent at diagnosis and resolved after a gluten-free diet.4–6 Bagnato et al reported a patient who presented with polyarthritis as in our case.7 The relationship of CD and arthritis is supported by the absence of other arthritis causing diseases and response to gluten-free diet. Hypogammaglobulinemia and lymphoma may associate with a flat jejunal biopsy and arthritis. Also gastrointestinal diseases such as Crohn and Whipple diseases, or causes of reactive arthritis, can lead to diagnostic difficulties. Sacroiliitis is not a known complication of CD but only a few studies have been reported. Recently, Vereckei et al8 reported that overt sacroiliitis may develop in 9.5% of patients with CD. The nature of sacroiliitis may vary from minimal erosive changes to overt clinical sacroiliitis. The pathogenesis of the arthritis is unclear in patients with CD. Genetic susceptibility, especially HLA-B8, B27, -DR3 positivity, and absorption of either immune complexes or gut-derived antigens may trigger humoral or cell-mediated response resulting in joint injury.6
CD should be kept in mind for the differential diagnosis of multiple gastrointestinal or extraintestinal conditions and serological tests for CD should be performed in all patients with arthritis of unclear etiology since joint involvement could be presenting symptoms of CD.
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