Gastrointestinal Disorders in Scleroderma

Scleroderma, meaning "hard skin," refers to a heterogeneous group of disorders in which patients often experience skin thickening and fibrosis. In the systemic form of the disease, systemic sclerosis (SSc), internal organ involvement and vasculopathy are prominent features, with gastrointestinal (GI) involvement especially common and affecting the esophagus in approximately 90% of patients. Symptoms and clinical disorders that reflect GI involvement in the cardinal pathogenic features of scleroderma (ie, vasculopathy, immune-mediated inflammation, and neuropathy) emanate from every segment of the GI tract: dysphagia and gastroesophageal reflux disease from the esophagus; gastroparesis and gastric antral vascular ectasia from the stomach; telangiectasia, pseudo-obstruction, and small intestinal bacterial overgrowth from the small intestine; constipation and colonic dilatation; and fecal incontinence due to thinning of the anal sphincters. Impaired motility is a cardinal feature reflective of atrophy of smooth muscle and scattered areas of fibrosis throughout the GI tract and results in impaired transit, stasis, and luminal dilation leading to complications such as small intestinal bacterial overgrowth, megacolon, and pneumatosis cystoides intestinalis. Symptoms often correlate poorly with the underlying GI pathology and functional impairment in SSc, and associations between GI manifestations, SSc disease features, and autoantibody titers are variable. High-quality SSc-specific evidence regarding the impact of various therapies on GI manifestations remains limited, and management recommendations for most GI manifestations are largely derived from experience in non-SSc patients. In caring for the patient with SSc with GI problems, a gastroenterologist should be aware of the particularities of clinical presentation and natural history of GI disease in this patient population, maintain diagnostic vigilance, and tailor their therapeutic approach mindful of the SSc disease process.