Background/Purpose: To examine the demography, clinical characteristics, features of intestinal lesions, treatment, and prognosis in patients with the intestinal Behcet’s disease currently followed at a university hospital in Tokyo especially focusing on the factors that correlated with additional immunosuppresive therapies to corticosteroid. 

Methods: The records of 39 patients with intestinal Behcet’s disease were retrospectively reviewed who were treated at the Teikyo University Hospital between August 1^st, 2011 and March 31th, 2012. We compared the well-controlled patients treated only with steroid or 5-ASA/SASP, with the poorly-controlled patients who required additional immunosuppressive drugs or anti-TNFa antibodies.

Results: The patients were consisted of 26 male and 13 female with the average age of 56.8 ± 13.1 years old. The mean age at onset of Behcet’s disease was 35.5 ± 11.2 years. They developed the intestinal lesions at mean age 41.3 ± 13.0 years. HLA-B51 or HLA-A26 were positive in 35.4% or 32.3%, respectively. Seventeen cases were complete Behchet’s and 22 were incomplete type. Almost all patients had oral ulcerations and skin lesions. Twenty five cases had arthritis, 7 had epididymitis. Vascular and central nervous system involvements were seen in 8 and 2 patients, respectively. The most frequent initial symptom for intestinal Behcet’s was abdominal pain (22 cases). Other initial symptoms were melena/bloody stool (16 cases) and diarrhea (9 cases), fever (5 cases), dysphagia (2 cases). The intestinal lesions existed in various lesions of the gastrointestinal tract including esophagus (3 cases) and small intestine (4 cases), ileo-cecal area (31 cases), ascending colon (7 cases), transverse colon (3 cases), descending colon (3 cases), sigmoid colon (2 cases), rectum (5 cases). They (28 cases) were treated with predonisolone of the average 32.5mg daily as the initial dosage. Thirty two patients were treated 5-ASA or SASP. Fourteen cases were added with methotrexate, and 3 cases with cyclosporine. Infliximab was administrated in 6 cases. In the patients who needed immunosuppressive drugs or anti-TNFa antibodies other than steroids, we found the significantly higher HLA-B51 positivity (42%) and higher CRP at the beginning of treatment (10.5±8.5 mg/dL). In addition, the poorly responded patients with corticosteroid and sulfasalazopyridine showed more frequent atypical intestinal lesions.

Conclusion: The restrospective review revealed that the requirement for the additional immunosuppresive therapies could have a linkage to the HLA-B51 in patietnts with intestinal Bachet’s disease.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/the-retrospective-review-of-39-intestinal-behcets-disease-focusing-on-the-requirement-for-the-immunosuppresive-drugs-other-than-corticosteroid/