Primary biliary cirrhosis: Article four in the series

Primary biliary cirrhosis (PBC) is a chronic cholestatic disease that usually affects middle-aged women. Its cause remains unclear, although there is considerable evidence to support an autoimmune etiology. The diagnosis is made on the basis of elevated alkaline phosphatase levels and increased titers of antimitochondrial antibodies. Treatment is aimed at slowing the progression of the disease, controlling symptoms, and managing hepatic failure. Several immunosuppressant and antifibrogenic agents have been tested, but have failed to demonstrate a survival benefit. Ursodeoxycholic acid is the most promising of the disease-modifying therapies. Orthotopic liver transplantation (OLT) offers the only definitive therapy for end-stage PBC and has shown encouraging results. Survival models have been developed to aid in the optimal timing and selection of patients for OLT, or order to make the best use of this limited resource.