The gastrointestinal tract, affecting more than 90% of patients, is the internal organ most frequently involved in systemic sclerosis. Any part of the gastrointestinal tract can be affected, from the mouth to the anus. Patients often experience reduced quality of life and impaired social life. Although only 8% have severe gastrointestinal involvement, mortality is high in those patients. Recent studies on the pathophysiology of the disease highlight new mechanisms to explain gastrointestinal dysmotility, but treatment remains symptomatic. This article reviews the pathophysiology of the gastrointestinal tract and discusses the investigation and management of the disease.
Key points
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A multidisciplinary approach with a gastroenterologist, nutritionist, and often a speech therapist is mandatory in all patients with severe gastrointestinal involvement.
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Oral cavity abnormalities are common in systemic sclerosis and can be severe.
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Gastroesophageal reflux may trigger or worsen interstitial lung disease.
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All patients with scleroderma should be screened for malnutrition.
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Treatment of fecal incontinence starts with optimization of the constipation treatment.
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Probiotics may be useful in patients with bloating and distension and small intestinal bacterial overgrowth.
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Well-powered prospective studies are needed to determine the effect of immunosuppressive treatment on the onset of gastrointestinal tract disease, especially in early systemic sclerosis.
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