Primary sclerosing cholangitis

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease with a male predominance that usually presents during the fourth decade of life. The disease incidence is 4-13 per million with a prevalence of 100-150 per million. Primary sclerosing cholangitis often leads to cirrhosis and the need for liver transplantation. The pathogeneses is unknown. Most patients have a slowly progressive course without spontaneous resolution. There is no effective therapy. Ursodeoxycholic acid (UDCA) leads to improvement in biochemical abnormalities but not in histology, cholangiographic appearance, or survival. Biliary strictures in PSC patients are treated with endoscopic balloon dilatation or biliary stent placement. Liver transplantation is the best treatment in end-stage PSC with 1-year survival greater than 90% and 5-year survival greater than 83%. The most serious complication of PSC is cholangiocarcinoma, which occurs in up to 10-15% of PSC patients. Further basic and clinical research is needed to improve the care of PSC patients.