Primary sclerosing cholangitis associated with inflammatory bowel disease

Abstract

Primary sclerosing cholangitis (PSC) is a rare chronic cholestatic liver disease characterized by inflammation of the intra and extrahepatic bile ducts with progression to cirrhosis. To the present, the etiology of PSC is not known. Although it is possible to establish a correlation with a genetic predisposition, it is an autoimmune disease, associated with ulcerative colitis and Crohn's disease. There is no proven medical treatment that prolongs survival in PSC, being liver transplantation the only curative treatment, with recurrence of 20% of cases within the first five years after transplantation.