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

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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.

Section snippets

Case selection

The death of 2 women from GIT SSc prompted an informal review of serious GIT involvement in SSc patients evaluated and treated at the Froedtert and Medical College of Wisconsin Rheumatology Clinic. Between 1995 and 2003 an additional 3 living cases were identified based on serious GIT involvement beyond the esophagus requiring diagnostic and therapeutic interventions for management. SSc patients with end-stage GIT involvement requiring total parental nutrition (TPN) were not included. These 5

Discussion and review of literature

Herein, we described 5 patients with SSc and severe GIT involvement, 4 with dcSSc and 1 with lcSSc. All but one had typical esophageal symptoms, mainly regurgitation and dysphagia. One patient had a distal esophageal stricture requiring dilation. Gastric symptoms were present in 2 patients though gastric dysmotility was documented in 3. Four patients had prominent small intestinal symptoms. Pseudoobstruction and bacterial overgrowth associated with poor nutrition were the cause of weight loss

Quality of life

Although GIT manifestations are common in patients with SSc, the impact on quality of life is underappreciated. As illustrated by case 2 and case 5, the presence of GIT symptoms had no correlation with severity of skin involvement. Both cases had regression of skin thickening when severe involvement of the colon and small intestine contributed to mortality and morbidity, respectively. Case 2 had GERD and dysphagia at diagnosis but developed colonic involvement 25 years later. Case 5 had onset

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