Deep vein thrombosis in Behçet's disease.

OBJECTIVE: We aimed to describe the epidemiological and clinical aspects of deep vein thrombosis (DVT) in Behçet's disease (BD) and to determine the patients at high risk for this complication. METHODS: Among 113 patients with BD according to the international criteria for classification of BD, those with DVT were retrospectively studied. The diagnosis of DVT was made in all cases using conventional venous angiography, venous ultrasonography and/or thoracic or abdominal computed tomography. Patients were divided in two subgroups according to the occurrence of DVT other than cerebral thromboses. The medical records of these patients were reviewed in order to investigate their past medical history and evaluate their response to the treatment prescribed. Clinical and genetic factors (HLA B51 and MICA 6) that might contribute to DVT were analysed by comparing patients with and without DVT. Results of our series were compared to those of other series in the literature. Statistical analysis was by Chi square with necessary correction and Fischer tests. RESULTS: Forty-four patients (38.9%) had deep vein thrombosis of various systems with 81 localisations. There were 40 men and four women (mean age 28.1 years; range 17-60). DVT appeared after the onset of disease with a mean delay of 3.8 years. In 6 cases, DVT revealed BD. When we evaluated the risk of DVT coexistence with other clinical findings and genetic factors (HLA B51 and MICA 6), we found a significant positive correlation with sex, and positive pathergy test. CONCLUSION: In our series, occurrence of DVT was significantly associated with male gender and positive pathergy test.

[Vena cava thrombosis in Behçet's disease. Analysis of a series of 10 cases]. / Thromboses caves dans la maladie de Behçet. Analyse d'une série de 10 observations.

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.