Abstract
Systemic sclerosis is a multisystem disease featured with autoimmunity and organ fibrosis. Although gastrointestinal (GI) tract involvement is common in patients with systemic sclerosis, colonic perforation is extremely rare. Benign pneumatosis intestinalis, a phenomenon more frequently seen in rheumatologic conditions, makes the diagnosis of colonic perforation even more challenging. We report a unique case of colonic perforation in a patient with chronic systemic sclerosis. This patient initially presented with mild abdominal pain and hematemesis. Urgent upper endoscopy was unremarkable and radiology showed stable pneumatosis intestinalis. Due to worsening abdominal pain, laparotomy exploration was performed and colonic perforation with transmural ischemic necrosis was found.
Highlights
Systemic scleroderma or systemic sclerosis is a chronic multisystem disease featured with generalized vasculopathy, activation of immune response, and progressive fibrosis of the skin and internal organs [1]
We report a unique case of colonic perforation in a patient with chronic systemic sclerosis
Systemic sclerosis is generally perceived as an orphan disease, its incidence was found to be comparable with the incidences of other well-known diseases such as esophageal cancer [6]
Summary
Systemic scleroderma or systemic sclerosis is a chronic multisystem disease featured with generalized vasculopathy, activation of immune response, and progressive fibrosis of the skin and internal organs [1]. Gastrointestinal (GI) tract involvement is very common in patients with systemic sclerosis and dysmotility is the primary manifestation [2]. GI Symptoms are usually nonspecific, including dysphagia, heartburn, nausea, vomiting, abdominal pain, and distention [3]. It is not uncommon to see chronic bowel dilation, pseudo-obstruction, and benign pneumatosis intestinalis on imaging, but these conditions are typically managed conservatively [2]. The bowel perforation is a rare but serious complication of systemic sclerosis with GI involvement [4, 5]. And accurate differentiation of true bowel perforation from benign pneumatosis intestinalis can be challenging
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