INTRODUCTION: Behcet’s Disease (BD) is a rare, chronic, inflammatory vasculitis that affects several organ systems. Gastrointestinal involvement occurs in 3 to 25% of patients, with prevalence of 1–2/1,000,000 in the United States. We present a case of a patient with BD developing abdominal pain, diarrhea, and weight loss. CASE DESCRIPTION/METHODS: 57-year-old man with known oral and genital BD presented with fever, 1-week 15lb weight loss, abdominal pain and non-bloody diarrhea. He had been on infliximab, colchicine and prednisone. Laboratory studies exhibited iron deficiency anemia and leukopenia. Abdominal CT showed wall thickening of terminal ileum with fat stranding consistent with terminal ileitis. Tuberculosis, Cytomegalovirus and Histoplasma on serum were negative. Colorectal surgery was consulted, and in light of refractory ileitis patient underwent laparoscopic right hemicolectomy with ileocolic anastomosis. Histopathology demonstrated acute ischemic necrosis with ileal ulceration. After surgery, he was continued on low dose prednisone and infliximab infusions with resolution of intestinal symptoms. DISCUSSION: Intestinal BD presents with abdominal pain, anorexia, vomiting, diarrhea, dyspepsia, and bloody/non-bloody stools. Mucosal ulcerations are thought to be the underlying factor behind the symptoms, likely secondary to a combination of vein and venule vasculitis and large vessel involvement leading to ischemia and subsequent infarction. Diagnosis can be challenging given clinical similarities with Inflammatory Bowel Disease (IBD), however findings of vasculitis on biopsy favors intestinal BD over IBD. Imaging modalities are useful in its diagnosis including barium swallow, CT, and MRI which demonstrate colonic wall thickening. Endoscopy often reveals large deep punched out ulcers with discrete margins usually in the ileocecal region. Treatment is similar to IBD, including medical and surgical modalities. First line agents are sulfasalazine, mesalamine and corticosteroids. Second line are infliximab and adalimumab. Colectomy is indicated if medical therapy fails or with presence of bowel perforation, fistulae or GI bleeding. After surgery, recurrence is seen up to 40–80% of cases, with 80% requiring re-operation. Keep in mind that patients with history of BD can develop colitis.Image 1.: Pre-operative CT showing the coronal sequence of images. Notice the circumferentially thickened, irregular terminal ileum in the right lower quadrant. There is secondary luminal narrowing and adjacent fat stranding compatible with transmural involvement.Image 2.: Pre-operative CT as above. Image series.
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