Primary Sclerosing Cholangitis

Primary sclerosing cholangitis (PSC) is a chronic cholestatic disorder characterized by inflammation and progressive obliterative fibrosis of the intrahepatic and/or extrahepatic bile ducts which can lead to cirrhosis and complications related to portal hypertension. PSC is strongly associated with inflammatory bowel disease (IBD), particularly ulcerative colitis (UC), as well as an increased risk of hepatobiliary and colorectal malignancy. PSC has several important subtypes, chiefly small duct PSC and PSC-autoimmune hepatitis (PSC-AIH) overlap. The pathophysiology behind PSC is poorly understood, but is likely a complex interaction between genes and the environment. Mounting evidence suggests key immunologic components involved in the development and progression of PSC. Currently, there is no effective medical therapy for PSC and liver transplantation (LT) provides PSC patients with excellent long-term outcomes. Aside from LT in appropriate patients, recognition and treatment of co-morbid conditions and cancer surveillance remain key components to the management of patients with PSC.