Characteristics of vascular involvement in Behçet's disease in Japan: a retrospective cohort study.

OBJECTIVES We analysed the clinical vascular characteristics of Behçet's disease (BD) patients in Japan. METHODS We retrospectively reviewed the clinical charts of 412 patients who fulfilled the 1987 Japanese criteria for BD and were treated in two University hospitals from July 1991 to December 2007. Patients with superficial thrombophlebitis were excluded, since it is categorised as a skin manifestation according to the Japanese criteria. RESULTS Twenty-six patients (6%) had large-vessel involvement. Mean ages at BD diagnosis and onset of vascular episodes were 39.7 and 41.6 years, respectively. Males predominated (62%). Arterial and venous lesions were found in 8 (31%) and 21 patients (81%), respectively, including 3 (12%) with both types. Pulmonary artery occlusion was the most common arterial lesion (n=5, 19%), followed by ascending aortic aneurysm (n=2, 8%). Limb deep vein thrombosis was the leading venous lesion (n=20, 77%). Cardiac complications (angina pectoris/aortic regurgitation) occurred in two patients. Gastrointestinal involvement was more frequent than in patients without vascular involvement (p<0.001); ocular involvement was less frequent (p<0.05). Only 3 patients (12%) required surgery. Patients received prednisone and immunosuppressants, including infliximab, for vascular and/or concurrent gastrointestinal involvement. Nine patients received warfarin, without bleeding complications. One patient died during the observation period, 4 days after surgery for an aortic aneurysm. CONCLUSIONS Frequency of vascular involvement in BD in Japan is lower than in other ethnic populations. Although one patient died during the observation, there was no fatal haemoptysis, even in patients receiving warfarin.