Budd-Chiari syndrome in the course of Behcet's disease: clinical and laboratory analysis of four cases.

摘要

OBJECTIVES: Budd-Chiari syndrome (BCS) is an uncommon manifestation of Behcet's disease (BD) characterized by hepatic venous outflow obstruction either within the liver or in the inferior vena cava (IVC). Clinical and laboratory findings, as well as treatment options and prognosis of four BD patients with BCS, were analyzed retrospectively. METHODS: Four male patients with BCS due to BD, ranging in age from 22 to 46 (median 24 years), were included in the present study. The presence of BCS was confirmed by Doppler ultrasonography and computed tomography and/or venography. Levels of protein C, protein S, antithrombin III, anticardiolipin antibodies and lupus anticoagulants were evaluated in all patients during the acute stage of BCS and both 6 months and 1 year after. Activated protein C resistance and prothrombin gene mutation were also determined in three patients. RESULTS: Two of the four patients with BCS had already been diagnosed with BD, while the other two were diagnosed with concurrent BD and BCS upon their admission to our clinic. All patients had deep venous thrombosis and superficial migratory thrombophlebitis prior to development of BCS. Case 1 died due to hepatic failure 1 month after his admission. The overall health state of the other three patients has remained good thanks to anticoagulation and immunosuppressive treatments. Three of these patients had one or more than one factor predisposing to thrombosis. CONCLUSIONS: The number of occluded hepatic veins and right hepatic vein involvement, as well as inferior vena cava thrombosis, can be deemed as a major determinant of prognosis. Although the presence of a thrombophilic condition may not be a factor for the clinical prognosis of BCS on its own, it may still necessitate a long-term anticoagulation for prevention of further thrombotic events.