Vena cava thrombosis in Behcet's disease. Analysis of a series of 10 cases

Vena cava thrombosis in Behcet's disease. Analysis of a series of 10 cases. Aim. - To study the clinical characteristics and the evolution of vena cava thrombosis (VCT) in Behcet's disease (BD), as well as their association wi th other severe symptoms. Patients and methods. - Among 121 ED, we selected those with VCT. Al patien ts fulfilled the diagnostic criteria of the international: study group of B ehcet's disease. Different clinical and paraclinical parameters were determ ined and compared with the remaining group of patients (not having VCT) wit h chi(2) test with Yates' correction. Protein C, protein S and antithrombin III and anticardiolipin antibody (aCL) levels were measured in 9 patients; anti-beta2-glycoprotein I antibodies (a beta 2GPI) were determined in 3 pa tients. Results. - Ten patients had a vena cava thrombosis(8.2%). They were all mal e with an average age of 35 years (range: 30-42). We had 3 cases of superio r vena cava thrombosis, 6 cases of inferior VCT, and one case of both. The average delay to diagnosis of the VCT from the date of the ED diagnosis was 4.5 years (range: 6 months-14 years), and in one case the thrombosis revea led the disease. All patients were clinically symptomatic and the installat ion of the symptoms were progressive and insidious in all cases, Six patien ts had Budd-Chiari syndrome and 4 had a phlebitis of a lower limb. Among al l the clinical caracteristics studied, only neurological manifestations was significantly higher in patients with VCT (p = 0.001). Protein C, protein S and antithrombin III levels were normal in all cases, One patients was po sitive for IgG aCL and no patient was positive for beta 2GPI. All our patie nts were treated by anticoagulation therapy and high-dose prednisone combin ed with intravenous cyclophosphamide in 5 cases. One patient died due to li ver failure. The 9 others are clinically improved (6 cases) or stable (3 ca ses) after an average 2.5 year course.