Primary biliary cirrhosis: clarifying the issues.

Abstract

Primary biliary cirrhosis is a disease of unknown etiology characterized by the destruction of small intrahepatic bile ducts, probably on an immunologic basis. The disease predominantly affects females. Serum biochemical tests reveal cholestasis, and a serum mitochondrial antibody is usually present. Other diseases, largely autoimmune, may be associated. Main extra- and intrahepatic bile ducts are patent. Treatment is symptomatic, and particular attention must be paid to the control of pruritus and to the prevention of fat-soluble vitamin and calcium deficiencies. Many immunosuppressant drugs have been tested but largely abandoned because of their failure to prolong life, inability to improve hepatic histology, or side-effects. Hepatic transplantation is the ultimate solution and should be considered sooner rather than later. The disease probably recurs in the transplanted liver.