Gastrointestinal complications of systemic sclerosis

Abstract

Systemic sclerosis is an autoimmune disease characterized by progressive skin thickening and tightness. Pulmonary interstitial fibrosis and kidney damage are the most important indicators for mortality; however, the gastrointestinal tract is the most commonly damaged system. Virtually all parts of the gastrointestinal (GI) tract can be involved, although the esophagus is the most frequently reported. The mechanisms that cause such extensive damage are generally unclear, but vascular changes, immunological abnormalities, excessive accumulation of collagen in the submucosa, smooth muscle atrophy and neuropathy may participate because these are the most common histological findings in biopsies and autopsies. Most patients with GI tract involvement complain about dyspepsia, nausea, vomiting, abdominal bloating/distension, and fecal incontinence. These symptoms are generally mild during the early stage of the disease and are likely ignored by physicians. As the disease becomes more advanced, however, patient quality of life is markedly influenced, whereby malnutrition and shortened survival are the usual consequences. The diagnosis for systemic sclerosis is based on manometry measurements and an endoscopy examination. Supportive and symptomatic treatment is the main therapeutic strategy; however, an early diagnosis is critical for successful management.

Keywords: Systemic sclerosis; Gastrointestinal tract; Manometry; Endoscopy; Diagnosis; Treatment

Core tip: Although often overlooked by clinicians, the gastrointestinal tract is the most commonly damaged system in patients with progressive systemic sclerosis. Virtually all parts of the gastrointestinal tract can be involved, although the esophagus is the most frequently reported. The mechanisms of gastrointestinal tract involvement have not been clarified; however, vascular damage, excessive accumulation of collagen, and immunological abnormalities may play a role because they are the most frequent histological findings in biopsies and autopsies. Non-specific symptoms, including dyspepsia, nausea, vomiting, and abdominal distension are common complaints. Although supportive and symptomatic treatment is the main therapeutic strategy for systemic sclerosis, early diagnosis is critical for improving patient prognosis.