Advances in therapy for primary sclerosing cholangitis

Primary sclerosing cholangitis is a chronic cholestatic liver disease frequently associated with inflammatory bowel disease whose pathogenesis is thought to be related to immunologic damage directed at bile duct epithelial cells. To date, there has been no totally effective therapy which achieves a complete biochemical and histologic remission. While some treatments have been associated with improvements in biochemical liver tests, none of the evaluated therapies have been convincingly shown to halt histologic progression of disease, prevent complications of portal hypertension, or prolong survival. Presently, clinical trials evaluating ursodeoxycholic acid, methotrexate, and cyclosporine are under way. Finally, in the end-stage of this disease, liver transplantation has been successful, and over 85% 3-year survival has been achieved at some of the leading liver transplant centers.