Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by fibrosing inflammation of the biliary tract. Bile duct obstruction, biliary cirrhosis, and hepatic failure may follow. The disease is often associated with chronic ulcerative colitis and must be distinguished from bile duct abnormalities arising from choledocholithiasis, previous biliary tract operations, congenital malformations, and cholangiocarcinoma. The widespread availability of endoscopic retrograde cholangiography beginning in the 1970s has led to the recognition of a progressively larger number of patients with PSC and has contributed to the rapid growth of knowledge about its clinical, radiographic, and histologic features. Advances have also been made in understanding the pathogenesis and treatment of PSC. Currently, the cause of PSC remains unknown, although alterations in immunologic function are most often implicated. No treatment has been proved to be beneficial in PSC; however, a number of exciting new developments have appeared on the horizon.
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