Recurrent primary sclerosing cholangitis: Clinical diagnosis and long‐term management issues

Key Concepts: 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. Liver Transpl. 12:S73–S75. 2006. © 2006 AASLD.