Severe gastrointestinal involvement in systemic sclerosis: report of five cases and review of the literature

Abstract

Objective: To review current literature on the gastrointestinal tract (GIT) manifestations of systemic sclerosis (SSc) and to report on 5 patients with severe gastrointestinal SSc.

Materials and methods: The clinical course and histopathology of 5 patients are described. A review of the medical literature registered in MedLine and PreMedLine databases from 1996 through mid-2004 was performed using the keywords systemic sclerosis and scleroderma and combining them with text words such as gastric, gastrointestinal, anorectal, colonic, and hepatic.

Results: All 5 patients had severe GIT involvement: 4 with diffuse cutaneous SSc (dcSSc) and 1 with limited cutaneous SSc (lcSSc). Autopsy results of 2 patients who died from severe malnutrition and aspiration pneumonia are presented. Literature review includes involvement from oral cavity to anus with varying degrees of severity. Most GIT manifestations result from dysmotility secondary to infiltration of the gastrointestinal wall with fibrous tissue and can cause life-threatening malabsorption and malnutrition. Diagnostic tests, pathology, and treatments of GIT SSc are reviewed.

Conclusions: GIT involvement in SSc can be severely debilitating and even life-threatening. Although morbidity is inevitable, appropriate supportive treatment can prolong survival.

Relevance: GI complications of SSc cause significant morbidity and mortality.