Isolated Hepatic Sarcoidosis Presenting as Non-Cirrhotic Portal Hypertension

Introduction: Sarcoidosis is the systemic formation of noncaseating granulomas. Hepatic involvement is well known and can present as abdominal pain, pruritus, hepatomegaly, or jaundice. Rarely, does it progress to portal hypertension or cirrhosis. Hepatic sarcoidosis in the absence of pulmonary involvement is seen in less than 10% of patients. Here we feature a case of isolated hepatic sarcoidosis discovered incidentally presenting as portal hypertension. Case Description/Methods: A 47-year-old male with past medical history of diabetes mellitus, hypertension, and alcohol use presented after a motor vehicle accident. Trauma workup was negative, although CT CAP incidentally noted scattered small pulmonary nodules, fatty liver, and splenomegaly with splenic varices. Follow up outpatient MRI showed hepatosplenomegaly and portal hypertension. Subsequent hepatic doppler revealed patent hepatic vessels and normal bile ducts. Initially, the liver findings were thought to be due to fatty liver disease given risk factors of metabolic syndrome and alcohol use of 6-8 beers per day. Labs were unremarkable except for mild thrombocytopenia with platelet count of 129 thou/cmm. Serologic workup was negative except for elevated smooth muscle antibody 1:160 and serum immunoglobulin G 1827 g/L suggesting autoimmune hepatitis. EGD noted small esophageal varices and isolated gastric varices. Ultimately, the patient underwent percutaneous liver biopsy, which showed noncaseating granulomas without significant fibrosis. There was no steatosis or features of autoimmune hepatitis. Stains for acid-fast bacilli and fungi were negative. Patient was diagnosed with hepatic sarcoidosis presenting as non-cirrhotic portal hypertension. Other organ involvement was not found. He was started on nadolol for prophylaxis of variceal bleeding. Treatment for sarcoidosis was not initiated given lack of clinical symptoms and normal liver enzymes. Discussion: Our case demonstrates the importance of considering hepatic sarcoidosis as a cause of portal hypertension. Liver involvement is common in sarcoidosis seen in 70% of cases, but significant complications including cirrhosis and portal hypertension are rare, about 3%. Non-cirrhotic portal hypertension as seen in our case is pre-sinusoidal and thought to be secondary to granuloma compression of the portal vein. Treatment is reserved for those with clinical symptoms with first-line therapy being glucocorticoids. Alternatives include ursodeoxycholic acid, immunosuppressants, and rarely liver transplant.Figure 1.: Specimen obtained from percutaneous liver biopsy with pathology showing foci of noncaseating granuloma.