Behçet's Disease-Related Budd-Chiari Syndrome Responding to Combination of Cyclophosphamide, Rivaroxaban and Corticosteroids

Abstract

culitic disease which is characterized by recurrent oral and genital aphtous ulcers, skin lesions, uveitis, arthritis and vasculitis.1 Thrombosis as a result of vasculitis and lower extremity deep vein involvement as the leading finding may be present. Budd-Chiari syndrome (BCS) is caused by obstruction of major hepatic veins. These patients may progress to liver failure and portal hypertension over time.2 BCS is related with BD in 5% of cases in western countries, while 9% in Turkey and 13% in Egypt.3,4 However, misdiagnosis or delay of diagnosis is common in patients with BD-related BCS.5 Some of the patients may be asymptomatic, while abdominal pain, jaundice, emesis, hepatomegaly and ascites are frequently present. In these patients, incomplete diagnostic criteria of BD should not delay the treatment. Here, we present a patient hospitalized for elevated liver enzymes and history of lower extremity thrombosis diagnosed later as BD-related BCS responding to combination therapy.