Venous Thrombosis and Cirrhosis Complicating Hepatic Sarcoidosis

Abstract

Sarcoidosis is a multisystem granulomatous disorder of unknown etiology. Hepatic involvement by granulomas is common but cirrhosis and portal hypertension are rare (each < 1%). We describe a patient with sarcoidosis involving the liver and bone marrow who presented with cirrhosis, portal hypertension, and thrombosis of the IVC, portal, and hepatic veins. A 33 year old Caucasian man was diagnosed 8 years ago with sarcoidosis involving only the liver and bone marrow. Liver biopsy at that time revealed chronic inflammation, fibrosis, and multifocal noncaseating granulomas with negative fungal and AFB cultures. Bone marrow biopsy showed multiple granulomas as well. The patient was treated with steroids and methotrexate. He presented again in 07/2005 with fever, edema, and splenomegaly. Labs showed wbc 2.1 K/mcL, hemoglobin 11 g/dL, platelets 94 K/mcL, total bilirubin 1.6 mg/dL, albumin 2.5 g/dL, alkaline phosphatase 229 U/L. Transaminases and INR were normal. The repeat bone marrow biopsy again demonstrated multiple granulomas. An abdominal CT scan revealed cirrhotic-appearing liver, recanalized paraumbilical vein, marked splenomegaly (22 cm), multiple venous collaterals, esophageal varices and thrombosis of the portal vein and IVC but no ascites. Additionally, Duplex ultrasound showed thrombosis of the right and left hepatic veins but patent middle hepatic vein. An IVC venogram showed a non-obstructing thrombus at the level of the right renal vein. The entire work up for a hypercoagulable state and other possible etiologies of chronic liver disease was negative. An EGD revealed grade 2 esophageal varices and portal hypertensive gastropathy. The patient was treated with prednisone, beta-blocker for esophageal varices, and Lovenox for the extensive thrombosis. Sarcoidosis with cirrhosis and extensive thrombosis of the IVC, portal, and hepatic veins is uncommon. Pathogenesis of cirrhosis and portal hypertension in patients with sarcoidosis is not well understood. Cirrhosis may be caused by ischemia of the liver parenchyma due to primary vascular injury by granulomatous phlebitis with or without thrombosis, or may be a result of bile duct destruction by granulomas (“biliary type” cirrhosis). Portal hypertension in sarcoidosis may occur as a complication of cirrhosis, as a result of portal granulomas compressing the small portal veins, or due to granulomatous phlebitis (± thrombosis) of the portal or hepatic veins.