Primary biliary cirrhosis: critical evaluation and treatment policies

Primary biliary cirrhosis is a disease where small intra-hepatic bile ducts are destroyed as a result of an immunological reaction, presumably by cytotoxic T-lymphocytes on biliary epithelium. Analogies can be made to the graft-versus-host reaction. Hepatic copper retention is secondary to cholestasis. A circulating mitochondrial antibody is virtually constant. A mitochondrial antibody, specific for primary biliary cirrhosis, has been identified. Liver biopsy appearances have been staged but there are difficulties because of the varying rte of evolution in different parts of the liver, the focal nature of the changes and the lack of correlation with the clinical picture. Other diagnostic criteria include female sex, raised serum alkaline phosphatase values and various associated diseases. Many patients are now diagnosed when presymptomatic and such patients, particularly with granulomas in the liver, may have a normal life expectancy. General therapeutic measures include control of pruritus and prevention of fat soluble vitamin and calcium deficiencies. There is no specific treatment but D-penicillamine is useful in the later stages and prolongs survival. The prognosis in those with progressive jaundice is very poor.