21. Primary Biliary Cirrhosis

  1. Eugene R. Schiff MD, MACP, FRCP2,
  2. Willis C. Maddrey MD, MACP, FRCP3 and
  3. Michael F. Sorrell MD, FACP4
  1. Stephen P. James MD

Published Online: 31 OCT 2011

DOI: 10.1002/9781119950509.ch21

Schiff's Diseases of the Liver, Eleventh Edition

Schiff's Diseases of the Liver, Eleventh Edition

How to Cite

James, S. P. (2011) Primary Biliary Cirrhosis, 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.ch21

Editor Information

  1. 2

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

  2. 3

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

  3. 4

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

Author Information

  1. Division of Digestive Diseases and Nutrition, National Institute of Diabetes & Digestive & Kidney Diseases, National Institutes of Health, Bethesda, MD, USA

Publication History

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

ISBN Information

Print ISBN: 9780470654682

Online ISBN: 9781119950509



  • Cholestasis;
  • cholangitis;
  • antimitochondrial antibody;
  • autoimmunity;
  • overlap syndrome;
  • ursodeoxycholic acid (UDCA)


Primary biliary cirrhosis (PBC) is an uncommon, chronic, cholestatic liver disease of unknown etiology characterized by necrosis of intrahepatic bile ducts and progressive portal fibrosis and cirrhosis. Autoimmune mechanisms have been implicated in pathogenesis, based on the presence of relatively specific antimitochondrial antibodies (AMAs) in 95% of patients, frequent association with other autoimmune syndromes, strong female predominance, and association with genes implicated in other autoimmune diseases. PBC may have a long, indolent, asymptomatic phase; when symptoms occur, they are nonspecific and include fatigue, pruritus, and jaundice. Diagnosis is based on the presence of typical biochemical features of cholestasis, high titer-positive AMAs, and the absence of evidence of diseases that mimic PBC and can be confirmed by the presence of typical liver biopsy histologic features. The only specific therapy that has been shown to alter the natural history of the disease is ursodeoxycholic acid. Other therapies are useful in managing symptoms and complications of the disease related to chronic cholestasis and cirrhosis. For patients with end-stage liver disease, the results of liver transplantation are excellent, although the disease may recur in a minority of patients.