Primary Sclerosing Cholangitis

There is no proven medical therapy for primary sclerosing cholangitis. The goal of management should be treatment of symptoms and complications of cholestasis, as well as attempts at treating the underlying disease process. In addition, efforts should be made to recognize and treat or prevent the known complications of primary sclerosing cholangitis, such as fat-soluble vitamin deficiency, osteopenia, dominant biliary strictures, and cholangiocarcinoma. Although some medical therapy has been shown to improve serum liver test or histology results, there has been no effect on survival or time to liver transplantation. However, preliminary data on high-dosage ursodeoxycholic acid have been encouraging. Liver transplantation remains the only effective treatment and is recommended for patients with end-stage liver disease and symptomatic portal hypertension, liver failure, and recurrent or intractable bacterial cholangitis.