Is Familial Mediterranean Fever a Possible Cofactor for Budd‐Chiari Syndrome?

Abstract

Budd-Chiari syndrome (BCS) is characterized by obstruction of hepatic venous outflow at any level of the hepatic veins or the inferior vena cava (IVC). Clinical presentation ranges from asymptomatic cases to fulminant hepatic failure or end-stage liver disease (1). Primary endophlebitis or congenital malformations may be responsible for BCS (1). Although several hypercoagulable states such as antithrombin III, protein C and S deficiency, factor V Leiden (FVL), and prothrombin gene 20210A mutations have been linked to BCS, recent data show that primary BCS is a multifactorial disease and the combination of several prothrombotic factors play a role in the development of thrombosis (1). Familial Mediterranean fever (FMF) is an autoinflammatory disorder characterized by episodic fever and polyserositis. Hypercoagulability has been recently reported in patients with FMF, yet thrombotic events were rarely reported in the literature. Although large vessel thrombosis in patients with FMF without amyloidosis is an unexpected event, an occurrence of superior vena cava thrombosis in a patient with FMF has also been reported (2-4). Our report is one of the few articles in the literature in which a major thrombotic event has occurred in the setting of FMF without amyloidosis.