Primary sclerosing cholangitis.

Abstract

Primary sclerosing cholangitis (PSC) is an inflammatory disease of unknown etiology. It affects the biliary system and is characterized by fibrosis and progressive obliteration of extrahepatic and intrahepatic bile ducts. Its frequent coexistence with antibody-mediated diseases, certain human leukocyte antigens, and altered lymphocyte mechanisms suggests a strong autoimmune and genetic etiology. Other conditions such as recurrent cholangitis, acquired immunodeficiency syndrome, previous bile duct surgery, bile duct anomalies, or biliary stone disease may result in similar clinical patterns and radiologic profiles. PSC can be asymptomatic and slowly progressive over years, or it may develop rapidly with an inexorable progression to cirrhosis and portal hypertension. Clinical and biochemical abnormalities may suggest PSC, but it is the characteristic radiological pattern that secures the diagnosis. Once viewed as a rare entity, the widespread use of radiological and endoscopic methods have increased physician awareness. There is no successful medical treatment for PSC. While traditional surgical approaches have been directed at the relief of cholangitis and biliary obstruction, liver transplantation has emerged as definitive therapy for patients with PSC complicated by cirrhosis or recurrent bouts of cholangitis.