[Primary biliary cirrhosis: therapy and prognosis]

Primary biliary cirrhosis (PBC) is thought to be an autoimmune disorder with additional liver injury being mediated by the subsequent cholestasis and accumulation of toxic bile acids. The combination therapy with ursodeoxycholic acid and colchicine is considered most effective and safe, among various therapeutic agents, such as corticosteroids, penicillamine, azathioprine and cyclosporine A for PBC patients. Several clinical trials using this combination therapy have shown significant improvement in symptoms and biochemical parameters, and a possibility of prolonging survival period. The survival of asymptomatic PBC patients may be more than 10 years. However, the clinical course may rapidly progress and show a poor prognosis, when jaundice occurs (bilirubin levels > 2 mg/dl). Liver transplantation remains the only choice of treatment at the end stage of the disease.