Evidence-based therapy of primary biliary cirrhosis

Primary biliary cirrhosis (PBC) is a disease which predominantly affects mi ddle-aged women and is characterized by destruction of the interlobular bil e ducts by chronic, often granulomatous, inflammation, This causes ductopen ia and consequent cholestasis. Progressive fibrosis leads to cirrhosis and eventual liver failure. The frequent association of other autoimmune diseas es and direct laboratory evidence of disturbed immune function suggest that PBC is an immune-mediated liver disease. Hence many clinical trials of the rapy have employed immunosuppressive drugs, Another approach to therapy has been to reduce the degree of liver damage secondary to the cholestasis by altering the intra-hepatic bile acid milieu. These very different approache s to treatment of PBC are reviewed. Eur J Gastroenterol Hepatol 11:607-615. (C) 1999 Lippincott Williams & Wilkins.