[Primary biliary cirrhosis and sarcoidosis. Association or unique disease?]

The incontestable simultaneous occurrence of primary biliary cirrhosis and sarcoidosis is reported in a 40 year-old woman. The diagnosis of primary biliary cirrhosis was based on the association of intrahepatic cholestasis, increased serum IgM, high titer of antimitochondrial antibodies and hepatic histologic lesions. In six years the latter passed from bile duct destruction with fibrosis to cirrhosis with absence of bile ducts. The diagnosis of sarcoidosis relied on the presence of bilateral hilar lymphadenopathies, pulmonary infiltrate, tuberculoid granuloma without necrosis in an enlarged cervical lymph node and increased serum level of angiotensin conversion enzyme improved by steroid therapy. Such an association, only three other cases of which have been reported, suggests a link between the two diseases, which could be two localizations of a same physiopathological process.