20. Primary Sclerosing Cholangitis

  1. Eugene R. Schiff MD, MACP, FRCP3,
  2. Willis C. Maddrey MD, MACP, FRCP4 and
  3. Michael F. Sorrell MD, FACP5
  1. Douglas L. Nguyen MD1,
  2. Nicholas F. LaRusso MD2 and
  3. Konstantinos N. Lazaridis MD1

Published Online: 31 OCT 2011

DOI: 10.1002/9781119950509.ch20

Schiff's Diseases of the Liver, Eleventh Edition

Schiff's Diseases of the Liver, Eleventh Edition

How to Cite

Nguyen, D. L., LaRusso, N. F. and Lazaridis, K. N. (2011) Primary Sclerosing Cholangitis, in Schiff's Diseases of the Liver, Eleventh Edition (eds E. R. Schiff, W. C. Maddrey and M. F. Sorrell), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781119950509.ch20

Editor Information

  1. 3

    Center for Liver Diseases and Schiff Liver Institute, University of Miami Miller School of Medicine, Miami, FL, USA

  2. 4

    Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, TX, USA

  3. 5

    University of Nebraska College of Medicine, Omaha, NE, USA

Author Information

  1. 1

    Center for Basic Research in Digestive Diseases, Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine, Rochester, MN, USA

  2. 2

    Department of Internal Medicine, Mayo Clinic College of Medicine, Rochester, MN, USA

Publication History

  1. Published Online: 31 OCT 2011
  2. Published Print: 9 DEC 2011

ISBN Information

Print ISBN: 9780470654682

Online ISBN: 9781119950509



  • Biliary epithelia;
  • cholestasis;
  • cholangiocarcinoma;
  • genetic predisposition;
  • liver transplantation;
  • inflammation;
  • pruritus


Primary sclerosing cholangitis (PSC) is a progressive, cholestatic liver disease defined by diffuse inflammation and fibrosis of the bile ducts and is associated with inflammatory bowel disease. PSC leads to obliteration of the biliary tree and ultimately biliary cirrhosis. PSC has been diagnosed more frequently in the past three decades because of routine liver biochemistry testing in clinical practice and widespread availablility of endoscopic retrograde cholangiopancreatography and magnetic resonance cholangiopancreatography. To date, the etiology of PSC remains unknown and effective medical therapy is not currently available. Patients with PSC have shortened life expectancy due to disease progression and associated co-morbidities such as cholangiocarcinoma and colon cancer. Orthotopic liver transplantation extends the live of patients with advanced stage PSC.