Severe gastrointestinal involvement in systemic sclerosis: Report of five cases and review of the literature
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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Disclaimer: This work is not supported by any pharmaceutical company