S5621 When Vasculitis Targets the Liver: Budd-Chiari Syndrome in a Patient With Behçet Disease

Introduction: Behçet's disease is a chronic multisystem inflammatory vasculitis that can cause thrombosis in blood vessels, including the hepatic veins, leading to Budd-Chiari syndrome (BCS). It is a life-threatening condition characterized by hepatic venous outflow obstruction. While Behçet's disease-related BCS is rare, it can significantly impact mortality. Case Description/Methods: A 22-year-old woman with no past medical history presented with bilateral lower extremity edema and abdominal fullness over the past week. She was not taking supplements or prescribed medications. Physical examination demonstrated painful ulcers in her mouth and genital region. Laboratory studies were significant for alkaline phosphatase 99 IU/L, total bilirubin 2.3 mg/dL, albumin 2.5 gm/dL, aspartate aminotransferase of 347 IU/L, alanine aminotransferase 242 IU/L and international normalized ratio was 2.3. Hypercoagulable testing for beta-2 glycoprotein antibodies, anticardiolipin antibodies, lupus anticoagulant and JAK2 V61 7F were unremarkable. Protein C and S testing were normal. Flow cytometry for PNH was also negative. A lower extremity doppler exhibited a deep venous thrombosis in the right popliteal vein. A contrast enhanced computed tomography abdomen demonstrated an enlarged liver measuring 22 cm craniocaudally, chronic occlusion of the inferior vena cava, iliac veins, and femoral veins with extensive collaterals throughout the abdominopelvic wall. She was found to have acute BCS of the hepatic veins. Interestingly, she also had a filling defect in the right ventricular outflow tract demonstrating a contiguous thrombus extending from the IVC and hepatic veins. Bone marrow biopsy was pursued that did not show evidence of a myeloproliferative neoplasm that could be contributing. She successfully underwent placement of a transjugular intrahepatic portosystemic shunt (TIPS). She was discharged on enoxaparin therapy and continues to do clinically well. Discussion: Although the pathophysiology is unknown, BCS is a vascular complication that can be associated with Behçet's disease. While Behçet's disease itself has a relatively low mortality rate (3%-4%), BCS in patients with Behçet's disease is associated with a much higher mortality rate, sometimes reaching 66%. The clinical signs of BCS are heterogeneous, with manifestations ranging from acute liver failure to being completely asymptomatic. Most patients have predisposing prothrombotic conditions, emphasizing the importance of a detailed work-up. With appropriate treatment, outcomes do improve, especially with early intervention and multidisciplinary care.