3220 Systemic Sclerosis Leading to Recurrent Small Bowel Pseudo-Obstruction

Abstract

INTRODUCTION: Systemic sclerosis (SSc) is a chronic systemic disease defined by early microvascular obliterative changes, early activation of T and B-lymphocytes, and extensive fibrosis of the skin and internal organs. Although gastrointestinal involvment of SSc is common, severe intestinal disease manifested by malabsorption, recurrent pseudo-obstruction, or need for parenteral nutrition carries a poor prognosis and is seldom seen. We present the uncommon case of a patient with severe scleroderma bowel causing debilitating disease. CASE DESCRIPTION/METHODS: A 76-year-old female with history of atrial fibrillation, systemic sclerosis, achalasia s/p Botox injections, and duodenal bulb ulcer presented to our institution with vomiting of blood-streaked emesis, epigastric abdominal pain, and distension for two days. She had multiple hospitalizations for presumed obstructions in the past treated medically. Endoscopy 3 months prior revealed a large cratered duodenal ulcer located at the duodenal sweep with recent stigmata of bleeding (Forrest Class IIa) treated with epinephrine. Upon evaluation the patient was afebrile and normotensive. Physical exam was significant for mild diffuse abdominal tenderness and no gross blood on rectal exam. Labs were remarkable for Hgb 11.9, MCV 92.8, lactic acid 1.0, and lipase within normal limits. CT A/P showed multiple dilated small bowel loops with air-fluid levels and a fluid-filled patulous esophagus that was unchanged. Trauma surgery was consulted and we managed the patient conservatively with bowel rest, IV fluids, and continuation of her PPI. Her clinical course improved and she was able to pass flatus and stool. We attributed her recurrent symptoms to intestinal pseudo-obstruction precipitated by scleroderma. DISCUSSION: SSc is a chronic autoimmune condition commonly affecting the gastrointestinal tract. Major manifestations of small intestinal involvement are due to reduced peristalsis, causing abdominal distension and pain from dilated bowel loops. Acute intestinal pseudo-obstruction is a medical emergency that warrants hospital admission. Most patients have spontaneous resolution with conservative measures like bowel rest, nasogastric decompression, and intravenous hydration but may require frequent readmissions. Surgery is discouraged due to risk of prolonged ileus or anastomotic failure although “venting” jejunostomy allows for air aspiration in chronic pseudo-obstruction. Recurrent intestinal pseudo-obstruction in these patients thus poses a therapeutic challenge.