Presentation and Outcome of Egyptian Patients with Primary Budd Chiari Syndrome (BCS) Related to Behçet’s Disease (BD)

Abstract

he aim beyond this study was to evaluate the presentation and outcome of Egyptian patients with primary Budd Chiari Syndrome (BCS) related to Behçet’s disease (BD). To fulfill such purpose, the characteristics of 39 BCS patients with BD diagnosed between May 2005 and 2016 December were compared to 232 BCS patients without BD. All the 39 patients with BCS and BD met the ICBD criteria for BD. BD patients were followed in department of Tropical medicine at Ain Shams university hospital. The diagnosis of BCS was based on confirmation of hepatic venous outflow tract obstruction based on imaging data: venous Doppler ultrasound, CT angiography and/ or magnetic resonance angiography (MRA) when required .Venous outflow obstruction due to right-sided heart failure and sinusoidal obstruction syndrome were not included. Data on demographic characteristics were collected during the study period (gender, age and geographic origin), clinical presentation of BCS (date of BCS, main symptoms, number of occluded veins), risk factors for thrombosis [proteins C, S and ant thrombin III deficiency R506Q mutation of factor V, G20210A mutation of prothrombin and C677T mutation of methylene-tetrahydrofolate-reductase (MTHFR) gene, plasma homocysteine level, anticardiolipin, tests for paroxysmal nocturnal hemoglobinuria and JAK2 V617F mutatio