Primary sclerosing cholangitis : Diagnosis and management

摘要: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease caused by progressive inflammatory destruction of intrahepatic and extrahepatic bile ducts, ultimately cirrhosis. PSC occurs primarily in patients with underlying ulcerative colitis affects young to middle-aged men. believed be an autoimmune mediated immune dysregulation genetic susceptibility. One possible mechanism for the development homing memory lymphocytes biliary tract. Cholangiography gold standard diagnosis PSC. The typical radiologic findings include multifocal strictures dilation involving or tract, both. Although no medical therapy has proved beneficial, variety agents have been tested, some which appear promising deserve further study. High-dose ursodeoxycholic acid may benefit slowing progression; multicenter placebo-controlled trial ongoing. Liver transplantation good option advanced PSC, although can recur after successful transplantation.