Background Behcet's disease is a systemic vasculitis disease with oral and genital aphthous ulcers, ocular involvements, skin manifestations, arthritis, gastrointestinal manifestations, neurogenic diseases and vascular involvements. Patients with Behcet's disease are known to distribute along the ancient Silk Road, including Japan.
Objectives We evaluate the clinical features of Behcet's disease in Japan.
Methods We retrospectively investigated 223 patients (108 males and 115 females) who fulfilled the International Criteria for Behcet's Disease (ICBD) from January, 2006 until May, 2015.We examined sex, onset age, disease type, clinical symptoms, laboratory data and medications.
Results Median age at diagnosis was 36.0±12.8 years old. Oral ulcers were the most common manifestation (98.2%), followed by genital ulcers (62.4%), ocular involvements (53.2%), erythema nodosum (53.2%), acneiform lesions (51.8%), arthritis (38.6%), gastrointestinal manifestations (25.1%), neurogenic diseases (9.0%), and vascular involvements (8.1%). The relationship of HLA and disease manifestations was studied in 123 patients (41.5% with HLA-B51 and 24.1% with HLA-A26). The frequency of acneiform lesions, ocular involvements and HLA-B51 was significantly higher in male, while genital ulcers and arthritis were significantly higher in female. Patients with ocular involvements showed a higher assosiation rate with neurogenic diseases and HLA-B51, and lower wih gastrointestinal manifestations. TNFα inhibitor (infliximab or adalimumab) were used for 66 cases (30%), and it could be continued for 1 year in 91%, and for 2 years in 83%.
Conclusions A higher incidence of gastrointestinal manifestations was observed in patients with Behcet's disease in Japan. Patients with ocular involvements showed a higher assosiation rate with neurogenic diseases, and lower wih gastrointestinal manifestation. Most patients could continue TNFα inhibitor safety and effectively.
Disclosure of Interest None declared