Background/Purpose: Behcet’s disease (BD) is characterized by recurrent oral aphthae and any of several systemic manifestations including genital ulcer, ocular disease, skin lesions, gastrointestinal involvement, neurologic disease, vascular disease, or arthritis. BD is more common along the ancient silk road, including Japan. We present the disease profile of Japanese BD patients at Kobe University Hospital.

Methods: We retrospectively investigated the clinical features of 127 patients (63 males and 64 females) who fulfilled the International Criteria for Behcet’s Disease (ICBD) and were treated in our hospital from April 2008 to May 2013. We assessed sex, age of onset, symptoms, complications, type of HLA and medications.

Results: Median age at diagnosis was 37.6 ±12.7 years old. Oral ulcers were the most common manifestation (97.6%), followed by genital ulcer (52.0%), ocular involvements (52.0%), papulopustular lesion (45.7%), erythema nodosum (43.3%), gastrointestinal manifestations (22.0%), vascular involvements (11.0%), and neurogenic diseases (5.5%). Ocular involvements were found to be significantly more frequent in males (male 63.5%, female 40.6%, P=0.01). The frequency of gastrointestinal manifestations was significantly higher in patients with vascular involvements (P=0.007), while patients with gastrointestinal manifestations showed a lower association rate with ocular involvements (P=0.001). Of the 127 BD patients, 2 patients were associated with spondyloarthropathy, one patient with giant cell arteritis and one with systemic sclerosis. The relationship of HLA and disease manifestations was studied in 54 patients (32.7% with HLA-B51 and 23.1% with HLA-A26). Ocular manifestations were significantly higher in patients with B51 than those without B51 (P=0.03). Colchicine was most commonly used for the treatment of BD (49.6%). TNFα inhibitor, infliximab, was used for patients with refractory ocular manifestations (n=21) and gastrointestinal manifestations (n=8). Only 3 patients had to be discontinued because of ineffectiveness or adverse events. The cumulative proportion of patients continuing infliximab therapy at 1 and 2 years was 95.8% and 84.3%, respectively. Pneumonia was the only severe adverse event.

Conclusion: A higher incidence of gastrointestinal manifestations was observed in Japanese patients with BD in our hospital. The frequency of gastrointestinal manifestations was significantly higher in patients with vascular involvements. Of the 127 BD patients, 25 patients (19.7%) were treated with infliximab, and the long-term infliximab treatment persistence rate was high.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-features-of-127-patients-with-behcets-disease-in-japan/