1597 An Unexpected Cause of Colonic Ulcers in a Young Chinese Male Resulting in a Total Colectomy—Intestinal Behcet's Disease

Abstract

INTRODUCTION: Behçet's disease (BD) is an uncommon immune-mediated inflammatory disorder. Gastrointestinal (GI) BD may affect any part of the GI tract but most commonly manifests in the ileocecal region. The diagnosis of GIBD is challenging and complications can be severe. CASE DESCRIPTION/METHODS: A 32-year-old Chinese male with HLA-B27 positive spondyloarthropathy was undergoing treatment with golimumab and methotrexate. He had a history of retinal vasculitis, erythema nodosum and was taking isoniazid for latent tuberculosis (TB). He was admitted for 1 day of right iliac fossa pain with occasional tongue ulcers but no fever or bloody stools. Abdominal computed tomography scan (CTAP) showed inflammatory changes in the ileocecal region. Colonoscopy with biopsies revealed an inflamed terminal ileum but no other colonic abnormalities; TB and cytomegalovirus biopsies were negative. He was discharged with antibiotics. He re-presented 4 weeks later with recurrent abdominal pain and 7-kg weight loss. CTAP showed worsening ileocecal thickening. The patient declined a colonoscopy only to be readmitted 5 days later for similar symptoms. Repeat colonoscopy showed large discrete round punched-out ulcers with overlying slough in the cecum, transverse, and left colon, with intervening normal mucosa. Biopsies showed extensive ulceration, acute inflammatory infiltrate with 1 non-caseating granuloma and intraluminal thrombi in submucosal arterioles/venules, raising the suspicion of gut vasculitis. Intravenous hydrocortisone and heparin were started. He developed worsening abdominal pain and a repeat CTAP showed pneumoperitoneum. Emergency surgery revealed multiple colonic perforations with feculent peritonitis necessitating total colectomy with end ileostomy. Histology revealed acute mucosal ulceration, areas of transmural necrosis, inflammation at perforation sites and luminal thrombi in the blood vessels around inflamed areas. A diagnosis of GIBD was made based on his constellation of symptoms. Treatment included prednisolone, cyclophosphamide, and he was later transitioned to azathioprine. DISCUSSION: In this immunosuppressed host with arthropathy presenting with abdominal pain, the key differentials of TB, Crohn's disease and GIBD are challenging to distinguish as there is significant clinical overlap. Endoscopic features may be helpful but are not pathognomonic of GIBD. This case highlights the diagnostic and management difficulties in GIBD and need for heightened awareness as outcomes in GIBD are potentially catastrophic.