Gastrointestinal manifestations of systemic sclerosis

Abstract

Introduction: Systemic sclerosis (SSc) is an autoimmune, multisystem disease of unknown cause characterized by diffuse fibrosis, degenerative changes, and vascular abnormalities in the skin, joints, and internal organs (especially the esophagus, lower GI tract, lungs, heart, and kidneys). After the skin, the most commonly involved organ is the gastrointestinal (GI) tract (in up to 90% of patients).Although GI disease is a cause of death in only a minority of patients with SSc, GI dysfunction is a major contributor to morbidity and they contribute considerably to impairment in quality of life. Materials and methods: We conducted a systematic review of observational studies that report GI problems in patients with scleroderma along with the Associated risk factors. Prevalence of each organ complication was extracted from studies in 2007-2015. Discussion results: Digestive involvement in systemic sclerosis is frequent and serious. Malnutrition, diarrhea, and constipation are some GI complications that can stem from scleroderma, and they contribute considerably to impairment in quality of life. Approximately 20% of people with scleroderma develop secondary Sjogren’s syndrome, a syndrome Associated with dry eyes and dry mouth. The most frequent visceral manifestation to be described was esophageal disease (70-90%). Oesophageal disorder is common with its main consequence: the occurrence of gastroesophageal reflux disease which could run into peptic erosive oesophagitis. Gastric involvement is rarely recognized but it is frequent in case of systematic investigation as well as small intestinal involvement which may provide a lot of complications: malabsorption, pseudoobstruction, bacterial overgrowth. At colonic level, anorectal involvement is frequent (50-70%) and leads to fecal incontinence and rectal prolapse. The symptomatic treatments must be systematic and improve the disease's overall prognosis. Although severe GI manifestations in SSc (defined as malabsorption, repeated episodes of pseudo-obstruction or severe problems requiring hyperalimination) are uncommon (8%), only 15% of such patients survived after 9 years of their diagnosis. Conclusion: Almost every part of the GI tract can be involved. GI involvement is often diagnosed after severe complications occurred and management can be difficult. At present, few specific therapeutic options are available for the treatment of these patients, but relief of symptoms is often possible with appropriate knowledge and support. It is therefore particularly important to identify, monitor and manage these patients carefully, with a view to minimalize further degeneration and maximalise quality of life.