Primary biliary cirrhosis, primary sclerosing cholangitis, and autoimmune cholangitis.

Primary biliary cirrhosis (PBC), and autoimmune cholangitis are presumed to be autoimmune cholestatic diseases, but the relevant antigens are unknown. Primary biliary cirrhosis is diagnosed by a positive serum mitochondrial antibody test. It usually affects women and has a very long course, culminating in liver transplantation or death. Ursodeoxycholic acid is probably the appropriate treatment. Primary sclerosing cholangitis (PSC) is marked by progressive destruction of extrahepatic and intrahepatic bile ducts. There is no specific diagnostic test or treatment. Cholangiocarcinoma is the dreaded complication and precludes liver transplantation, the only chance of a cure. Autoimmune cholangitis overlaps PBC and autoimmune chronic hepatitis. It is a rare condition, resembling PBC but with a negative serum mitochondrial antibody test; however, serum antinuclear antibodies and smooth muscle antibodies are present in high titers.