MADRID—For patients with significant gastrointestinal (GI) manifestations of scleroderma, the effect on quality of life and longevity itself can be dramatic. A recent study of two multi-center cohorts of patients with scleroderma who demonstrated features of severe GI involvement showed these patients have worse physical and mental health-related quality of life and a greater than twofold increase in the risk of death compared with those without severe GI disease. In the study, severe GI involvement was defined as malabsorption, hyperalimentation, pseudo-obstruction and/or ≥10% weight loss in association with the use of antibiotics for bacterial overgrowth or esophageal stricture.1
With this in mind, the 2019 European Congress of Rheumatology (EULAR), June 12–15, featured a Challenges in Clinical Practice session titled, Lights at the Ends of Both Tunnels? Advances in GI Involvement in Systemic Sclerosis. The session provided insights from patient cases, and the expert presentations helped shed light on this important subject.
The Big Picture
Carina Mihai, MD, PhD, senior rheumatologist at the department of rheumatology at Zurich University Hospital, Switzerland, provided an overview of the spectrum of GI tract involvement in scleroderma. In describing the pathogenesis of these issues, she noted the combination of progressive fibrotic changes in the GI tract along with neuromuscular dysfunction create what can be thought of as a traffic jam in the gut with resulting dysmotility, dilation of the intestine, malabsorption and, in some cases, malnutrition.
Esophageal hypoperistalsis can manifest as dysphagia or regurgitation, and nearly every patient with scleroderma will describe some degree of gastroesophageal reflux symptoms. In cases of delayed stomach emptying, patients may note bloating, early satiety, nausea and vomiting. Small intestinal hypomotility can also lead to bloating and constipation. Some patients will have features of intestinal pseudo-obstruction and possible small intestinal bacterial overgrowth, which may actually manifest with diarrhea. Likewise, colon hypomotility can result in constipation, while anorectal dysfunction can lead to incontinence, a symptom patients may be hesitant to discuss with clinicians unless directly questioned about it.
To assess for GI tract involvement in scleroderma patients, Dr. Mihai advocated for the use of the UCLA Scleroderma Clinical Trial Consortium Gastrointestinal Tract 2.0 (SCTC GIT 2.0) questionnaire. This 34-item questionnaire has seven multi-item scales that include reflux, distension/bloating, diarrhea, fecal soilage, constipation, emotional wellbeing, social functioning and a total GIT score. Dr. Mihai noted this tool provides a thorough and comprehensive assessment of patient symptoms and also helps capture an overview of the nature and severity of these symptoms.
