Background/Purpose: Recent studies suggest that coeliac disease (CD) affects 4-7% of Scleroderma (SSc) patients. This association however has not been well characterized in a large cohort of patients. We evaluate the key clinical features, nutritional complications, and clinical response after gluten free diet in SSc patients with CD (SSc-CD), compared with a group of SSc patients with gastrointestinal (GI) symptoms and negative coeliac antibodies.

Methods: This is a retrospective study of well-characterised cohort of 1920 SSc patients from 2008 to 2013 with data collected through clinical database and patient records including all SSc patients diagnosed with CD by duodenal biopsy (n=12), and 96 of 236 SSc patients with GI symptoms (chronic diarrhoea, bloating, constipation, discomfort, dysphagia, heartburn and faecal incontinence) and negative coeliac antibodies. 4 patients in the SSc-CD group had confirmed positive coeliac antibodies. Key demographic and clinical features including small bowel symptoms (chronic diarrhoea, bloating, constipation and discomfort) were examined.

Results: The prevalence of CD with positive coeliac antibodies in our SSc cohort with GI symptoms was 4/236 (1.69%). 92.6% were female, with median (interquartile range, IQR) of age at SSc onset of 45.0 (33.0 – 55.7) years, and 49.3 (41.0 – 55.7) years at CD diagnosis. 18.5% patients had diffuse subset and 66.7% of the patients were Caucasians. 33 (30.6%) had an overlap with another systemic autoimmune disease (17 had polymyositis-dermatomyositis, 8 rheumatoid arthritis, 5 Sjögren’s syndrome, 3 others). A higher frequency of psoriasis was identified in 25% of SSc-CD patients compared with 2.1% (p=0.009) in negative-coeliac antibodies patients. SSc-CD patients had increased frequency of myopathy (33.3% vs 10.4%, p=0.04), and anti-Pm/Scl antibody (25.0% vs 3.1%, p=0.01). 88% of entire cohort had small bowel symptoms with no statistical differences between the two groups. Chronic diarrhoea was the most common symptom in 62.0% of patients. Upper endoscopy was performed in all SSc-CD patients and in 41.7% with negative-coeliac antibodies, with no statistical differences in frequency of esophagitis, gastritis, gastric antral vascular ectasia or macroscopic duodenitis. There were no differences in the prevalence of bacterial overgrowth or requirement for enteral/parenteral nutrition. Among all patients 44.4% had vitamin D deficiency, 40.7% iron deficiency and 7.4% osteoporosis. After gluten-free diet all SSc-CD patients had an improvement of small bowel symptoms. 50% of these patients achieved complete remission and the symptoms recurred in the remaining half with median (IQR) 5.0 (2.5 – 8.25) years. There was significant improvement in chronic diarrhoea (75% to 41.7%, p=0.04), abdominal distension or bloating (50% to 16.7%, p=0.04), and in weight loss (33.3% to 0%, p=0.04).

Conclusion: The clinical presentation of CD may be indistinguishable to SSc-GI disease and our study indicates that CD in this subgroup of SSc patients with GI involvement is no more common than the general population. The positive response to gluten-free diet suggests that screening for CD in selected SSc patients may be helpful.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/coeliac-disease-in-scleroderma-clinical-features-frequency-and-impact-of-screening-in-scleroderma/