TY - CHAP M1 - Book, Section TI - Chapter 52. Primary Sclerosing Cholangitis A1 - Greenberger, Norton J. A1 - Saltzman, John R. A2 - Greenberger, Norton J. A2 - Blumberg, Richard S. A2 - Burakoff, Robert PY - 2012 T2 - CURRENT Diagnosis & Treatment: Gastroenterology, Hepatology, & Endoscopy, 2e AB - Progressive inflammatory, sclerosing, and obliterative disease of the extrahepatic or intrahepatic bile ducts, or both.Disproportionate elevation of serum alkaline phosphatase (4–10 times normal) is seen in almost all patients.MRCP or ERCP shows multifocal stricturing of the intrahepatic and extrahepatic bile ducts.Small duct disease is diagnosed by liver biopsy.Liver biopsy findings that are highly specific include fibrous obliteration of small bile ducts with concentric replacement by connective tissue in an "onion skin" pattern.Patients have an 8–15% lifetime risk of developing cholangiocarcinoma, and it is often difficult to distinguish a dominant stricture from a cholangiocarcinoma. SN - PB - The McGraw-Hill Companies CY - New York, NY Y2 - 2024/03/29 UR - accessmedicine.mhmedical.com/content.aspx?aid=55962059 ER -