Evidence-based therapy of primary biliary cirrhosis.
Primary biliary cirrhosis (PBC) is a disease which predominantly affects middle-aged women and is characterized by destruction of the interlobular bile ducts by chronic, often granulomatous, inflammation. This causes ductopenia and consequent cholestasis. Progressive fibrosis leads to cirrhosis and eventual liver failure. The frequent association of other autoimmune diseases and direct laboratory evidence of disturbed immune function suggest that PBC is an immune-mediated liver disease. Hence many clinical trials of therapy 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 approaches to treatment of PBC are reviewed.
Department of Medicine, The Toronto Hospital, University of Toronto, Ontario, Canada.
E J
EJ
Heathcote
D001379:Azathioprine / Q000627:therapeutic use; D001647:Bile Acids and Salts / Q000627:therapeutic use; D002699:Chlorambucil / Q000627:therapeutic use; D003078:Colchicine / Q000627:therapeutic use; D016572:Cyclosporine / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D019317:Evidence-Based Medicine; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008105:Liver Cirrhosis, Biliary / Q000188:drug therapy*; D008727:Methotrexate / Q000627:therapeutic use; D011239:Prednisolone / Q000627:therapeutic use; D016896:Treatment Outcome
D001647:Bile Acids and Salts; D007166:Immunosuppressive Agents; D002699:Chlorambucil; D016572:Cyclosporine; D011239:Prednisolone; D001379:Azathioprine; D003078:Colchicine; D008727:Methotrexate