Duodenal Behcetʼs Syndrome Successfully Treated with Azathioprine

Abstract

Behcet's Syndrome is a multisystem inflammatory disorder characterized by recurrent oral and genital ulcers. Ocular, joint, CNS, and GI involvement are all common, but GI lesions are typically resistant to medical therapy. We present a case of deep duodenal ulcers in a 44 year-old female successfully treated with azathioprine. A 44 year-old A.A. female with a history of Behcet's syndrome presented with 3 months of abdominal pain, nausea, and vomiting unresponsive to treatment with rabeprazole and sucralfate. Her symptoms were worse after eating. She denied any hematemasis, melena, hematochezia, diarrhea, or constipation. Medical history showed Behcet's diagnosed as a teenager. Family history showed a father with stomach cancer at 38. There was no history of NSAID use. On exam, multiple aphthous ulcers were seen in the oropharynx. Abdomen was soft and obese with moderate epigastric tenderness. Ophthalmologic, joint, and neurologic exam were all normal. Basic labs and CT of the abdomen were all normal. EGD showed multiple superficial erosions in the antrum and proximal esophagus. Two 1.5×3cm ulcers were seen in the D2 portion of the duodenum. Biopsies showed a non-specific “fibrinopurulent exudate consistent with ulcer.” Antral biopsies were negative for H. pylori. Serum gastrin and a SBFT were normal. Over the next 6 months her symptoms worsened despite the continuation of PPI therapy and the addition of hydroxychloriquine by her rheumatologist. She required large doses of narcotic agents in order to tolerate any food and, despite this, she lost 45 pounds and was forced to go on disability. Subsequent EGDs showed persistence of her large duodenal ulcers and small antral ulcers. Azathioprine 2.5mg/kg was started. Six weeks later, EGD showed resolution of the small antral ulcers and partial resolution of the duodenal ulcers. Subsequently, her symptoms resolved. Four months later, she has gained 20 pounds and no longer requires pain medications. Discussion: GI involvement of Behcet's is variable (2–50% of cases, largely dependent on geographic area of interest). Ileocecal and colonic involvement is most common, and intestinal lesions are usually resistant to medical treatment. There have been two previous case reports of duodenal involvement, each requiring a surgical intervention. A review of 136 Japanese surgical cases showed duodenal involvement in 2 cases. To our knowledge, this is the first report of duodenal Behcet's with successful medical treatment.