Primary sclerosing cholangitis

Abstract

Primary sclerosing cholangitis is a chronic cholestatic disease characterized by periductal inflammation of intrahepatic and extrahepatic bile ducts with obliterative fibrosis and duct loss. Significant loss of bile ducts leads to interference with bile flow and ultimately results in cirrhosis of the biliary type. Aside from viral hepatitis and alcoholic liver disease, primary sclerosing cholangitis is one of the most common indications for liver transplantation. Although there is no known beneficial therapy for these patients, research aimed toward better understanding the pathogenetic mechanisms and clinical trials with promising new agents are ongoing.