A Case of Sarcoidosis with Liver Cirrhosis Associated with Hepatopulmonary Syndrome

Abstract

When a 59-year-old woman visited our hospital in June 2003, she complained of having slight exertional dyspnea since December 2002. She had bilateral hilar lymphadenopathy and bilateral reticular shadows at lower lung with high 4/8 ratio in BAL fluid. Serum ACE level and PPD skin test were negative. She was diagnosed as having sarcoidosis. Hepatic dysfunction was also found. During follow-up without treatment in our outpatient department, exertional dyspnea gradually worsened and marked hypoxemia was shown. She was admitted to our hospital in June 2006. Chest CT showed bilateral basilar reticular opacities which were the same as CT at the first visit, but abdominal CT showed gastric varices and liver cirrhosis. Blood examination showed hepatic dysfunction but viral hepatitis was ruled out, and primary sclerosing cholangitis, primary biliary liver cirrhosis, and autoimmune hepatitis were negative serologically. We performed a liver biopsy which showed liver cirrhosis and non-caseating epithelioid cell granulomas. In addition, we found extrapulmonary accumulation in pulmonary blood flow scintigraphy and diagnosed it as hepatopulmonary syndrome with liver cirrhosis and non-caseating epithelioid cell granulomas, because no shunt was found.