Aim: To study the clinical characteristics and the evolution of vena cava thrombosis (VCT) in Behçet's disease (BD), as well as their association with other severe symptoms.
Patients and methods: Among 121 BD, we selected those with VCT. All patients fulfilled the diagnostic criteria of the international study group of Behcet's disease. Different clinical and paraclinical parameters were determined and compared with the remaining group of patients (not having VCT) with chi 2 test with Yates' correction. Protein C, protein S and antithrombin III and anticardiolipin antibody (aCL) levels were measured in 9 patients; anti-beta 2-glycoprotein I antibodies (a beta 2GPI) were determined in 3 patients.
Results: Ten patients had a vena cava thrombosis (8.2%). They were all male with an average age of 35 years (range: 30-42). We had 3 cases of superior 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 BD diagnosis was 4.5 years (range: 6 months-14 years), and in one case the thrombosis revealed the disease. All patients were clinically symptomatic and the installation of the symptoms were progressive and insidious in all cases. Six patients had Budd-Chiari syndrome and 4 had a phlebitis of a lower limb. Among all the clinical characteristics 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 positive for IgG aCL and no patient was positive for a beta 2GPI. All our patients were treated by anticoagulation therapy and high-dose prednisone combined with intravenous cyclophosphamide in 5 cases. One patient died due to liver failure. The 9 others are clinically improved (6 cases) or stable (3 cases) after an average 2.5 year course.