Curbside Consultation of the Liver: 49 Clinical Questions

Mitchell L. Shiffman, MD

Question 33: Is Ursodeoxycholic Acid Effective For The Treatment Of Primary Sclerosing Cholangitis? (continued)

B. Marie Reid, MD; Velimir A. Luketic, MD

Primary sclerosing cholangitis (PSC) is a progressive cholestatic liver disease characterized by inflammation and obliterative fibrosis of the intrahepatic and extrahepatic biliary tree. The causes for the initial bile duct injury remain largely unknown. Proposed mechanisms include genetic predisposition, immunologic abnormalities, bacterial proteins from the enterohepatic circulation, chronic viral infections, and ischemia. Progressive disease, however, is thought to be due to the development of cholestasis and retention of hydro-phobic bile salts within the liver. Bile salt–mediated…