Treatment of primary biliary cirrhosis and primary sclerosing cholangitis: use of ursodeoxycholic acid.

Considerable progress has been made in the management of cholestatic liver diseases during the past decade. Various therapeutic agents have been proposed and evaluated for treatment of patients with primary biliary cirrhosis and primary sclerosing cholangitis. These treatments include ursodeoxycholic acid plus immunosuppressive and anti-inflammatory drugs such as glucocorticoids, azathioprine, colchicine and methotrexate. Although these two diseases are grouped together as chronic cholestatic liver diseases, there are important differences between them, particularly with respect to response to treatment. Primary biliary cirrhosis responds much better to medical treatment. Ursodeoxycholic acid has emerged as the most commonly used medication in the treatment of these diseases. Ursodeoxycholic acid therapy is safe and has been associated with improvement of biochemical test results for liver function in patients with primary biliary cirrhosis and primary sclerosing cholangitis. However, questions remain about the long-term efficacy of the drug in halting histologic progression, although ursodeoxycholic acid does improve survival without the need for liver transplantation after 4 years of treatment in patients with primary biliary cirrhosis. Ursodeoxycholic acid is unproven in the treatment of primary sclerosing cholangitis.