Systemic Sclerosis in Small Bowel

Abstract

e t s P t p A man with systemic sclerosis was admitted with progressive abdominal distention and vomiting. Previusly, he had recurrent episodes of vomiting. There was no istory of abdominal surgery, and a recent colonoscopy was nremarkable. Physical examination showed a tympanitic, disended abdomen with hypoactive bowel sounds. A digital rectal xamination revealed low anal sphincter tone. Laboratory inestigations indicated a total white blood cell count of 0,930/ L and mild hypoalbuminemia (3.5 g/dL). Liver and enal function was normal, and urinalysis showed no protein. A lain abdominal radiograph and computed tomography reealed the dilation of the entire small intestine (Figure A). asting and fluid supplementation relieved his symptoms. Upper endoscopy revealed reflux esophagitis. Double-balloon ndoscopy by oral route revealed a narrow separation between ircular folds of the small intestine (Figures B and C). Villous tructure was normal. A biopsy specimen from the jejunum howed only mild chronic inflammation. He received a diagnois of systemic sclerosis–related pseudo-obstruction. The paient’s symptoms improved with prokinetic drugs for motility isturbance and antibiotics for bacterial overgrowth. Systemic sclerosis is a multisystem disease characterized by xcess deposition of connective tissue in skin and internal rgans. Gastrointestinal involvement is the most frequent inernal organ complication of systemic sclerosis, with more than 0% of patients experiencing a change in gastrointestinal funcion.1 Impairment in bowel motility leads to stasis of luminal contents and secondary bacterial overgrowth. The European League Against Rheumatism recommends prokinetic drugs for motility disturbance and antibiotic rotating therapy for bacterial overgrowth or malabsorption in systemic sclerosis.2 Typical ndoscopic appearance of small bowel manifestation of sysemic sclerosis is narrow separation between circular folds depite bowel dilation. This sign is referred to as hide-bound. redominant muscle atrophy and collagenous replacement of he outer longitudinal layer relative to the inner circular layer robably account for this characteristic appearance.3