2104 Treatment of Ulcerative Colitis Pan-Enteritis Following Colectomy With Cyclosporine

Abstract

INTRODUCTION: Extensive enteritis following colectomy for ulcerative colitis is a rare disease phenotype that is often difficult to treat. Surgical intervention can be prevented with initiation of steroids and calcineurin inhibitors. CASE DESCRIPTION/METHODS: An 18-year-old female with ulcerative colitis status post colectomy and restorative ileoanal anastomosis presented with bloody diarrhea two months after her ileostomy closure. An EGD and pouchoscopy demonstrated active inflammation in the duodenum and distal 60 cm of small bowel. Pathology revealed acute and chronic enteritis without evidence of cytomegalovirus. Despite prompt initiation of corticosteroids, blood transfusions, and parenteral nutrition, weight loss, diarrhea and anemia persisted. Brief ICU care was required for hypotension and worsening anemia. Intravenous cyclosporine (CyA) was initiated, maintaining a goal range of 300-400 ng/mL. Within 48 hours of cyclosporine salvage therapy, bloody diarrhea, enteral intake and anemia improved. She was discharged on oral prednisone taper and oral CyA, with plans to initiate azathioprine for maintenance of remission. DISCUSSION: Ulcerative colitis is defined as inflammation of the colon and rectum only. However, a variant of small bowel inflammation, referred to as ulcerative colitis- related pan enteritis, has been recently identified, with symptoms of nausea, abdominal pain, and bloody diarrhea typically occurring after several months after colectomy. Although the pathogenesis is unknown, some have hypothesized that massive cytokine mediated inflammatory response may continue after colectomy. Endoscopy and pathologic examination are essential for diagnosis with evidence of superficial, continuous ulcerative mucosal inflammation in the stomach and/or small bowel. Lack of aphthous ulcers, skip lesions and strictures can distinguish this entity from Crohn’s disease. The majority of patients will respond to intravenous corticosteroids, but use of calcineurin inhibitors in refractory cases has been reported. Long term management remains undefined, but a recent case series has reported success with use of azathioprine to maintain remission. We report a rare case of ulcerative colitis pan enteritis refractory to corticosteroid therapy, but responsive to intravenous CyA. This should be considered in the differential diagnosis for patients with signs of small bowel inflammation after a colectomy for ulcerative colitis. Prompt recognition and treatment can prevent unnecessary surgical interventions.