TY - CHAP M1 - Book, Section TI - Primary Sclerosing Cholangitis A1 - Greenberger, Norton J. A1 - Saltzman, John R. A2 - Greenberger, Norton J. A2 - Blumberg, Richard S. A2 - Burakoff, Robert Y1 - 2016 N1 - T2 - CURRENT Diagnosis & Treatment: Gastroenterology, Hepatology, & Endoscopy, 3e AB - ESSENTIALS OF DIAGNOSISProgressive 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.Magnetic resonance cholangiopancreatography (MRCP) or endoscopic retrograde cholangiopancreatography (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 - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/28 UR - accessmedicine.mhmedical.com/content.aspx?aid=1119991547 ER -