1. Primary sclerosing cholangitis (PSC) in the nontransplant setting is a chronic, progressive liver disease characterized by diffuse stricturing of the biliary tree, cholestatic liver enzymes, and a compatible liver biopsy. 2. Cholangiography reveals irregularity of the bile duct wall, strictures, beading, and diverticula. 3. The typical biopsy reveals inflammation and fibrosis of the interlobular and septal bile ducts, often with obliteration or biliary-type cirrhosis. 4. The precise pathogenetic mechanism remains elusive but is assumed to be an autoimmune phenomenon. 5. For patients with end-stage complications of PSC, such as liver failure, recurrent bacterial cholangitis, and intractable pruritus, liver transplantation is an acceptable treatment option.
Copyright 2006 AASLD.