Behçet's disease complicated with thrombosis: a report of 93 Chinese cases

To investigate the clinical features of Behçet's disease (BD) complicated with thrombosis. Medical records of patients with BD at Peking Union Medical College Hospital from 1993 to 2013 were reviewed to identify thrombosis. Of the 766 patients with BD, 93 patients (16 female and 77 male) developed thrombosis. The most common thrombosis was extremity vein thrombosis (86.0%), including deep vein thrombosis (n=78) and superficial thrombophlebitis (n=4). The other thrombosis types associated with BD in descending frequency of order were: vena cava thrombosis (30.1%), pulmonary thromboembolism (15.1%), cerebral venous thrombosis (CVT) (12.9%), intracardiac thrombosis (8.6%), Budd-Chiari syndrome (7.5%), and renal vein thrombosis (4.3%), etc. Venous thrombosis is more frequent than arterial thrombosis, and most of patients (94.6%) experienced multiple thrombosis. A male predominance of extremity vein thrombosis and positive pathergy test, and a female predominance of CVT and genital ulcers were noted. All of these patients exhibited active disease during the emergence of thrombotic events. After treating with glucocorticosteroids, immunosuppressants, and/or anticoagulants, the thrombosis resolved in 89 patients. Three patients died from aneurysm rupture, myocardial infarction and Budd-Chiari syndrome, respectively. One patient with septic shock discontinued therapy during follow-up. Thrombosis in BD patients is male predominance, mainly multiple and venous thrombosis is more common. Active disease patients are prone to thrombosis, which suggest the key role of immunosuppressive therapy for the complication.