Primary Biliary Cirrhosis

From Pathogenesis to Clinical Treatment

  • Keith D. Lindor
  • E. Jenny Heathcote
  • Raoul Poupon

Table of contents

  1. Front Matter
    Pages i-xv
  2. Natural history and pathogenesis

    1. Front Matter
      Pages 1-1
    2. M. F. Bassendine
      Pages 11-18
    3. M. E. Gershwin, C. T. Migliaccio, J. Van De Water, R. L. Coppel
      Pages 40-52
    4. C. D. Howell, J. Li, W. Chen
      Pages 53-63
    5. D. Schuppan, E. G. Hahn
      Pages 64-75
    6. W. R. Kim, E. R. Dickson
      Pages 76-83
  3. Management of primary biliary cirrhosis

  4. Back Matter
    Pages 173-176

About this book


The condition of prolonged obstructive jaundice with patent bile ducts was first described in 1851 by Addison and Gull of Guy's Hospital, London. The term primary biliary cirrhosis (PBC) was defined in 1950 by Ahrens and colleagues of the Rockefeller Institute, New York. The condition was considered rare but this changed in 1965 with the discovery of a definitive diagnostic serum mitochondrial antibody test and the recognition that a raised serum alkaline phosphatase value, often discovered incidentally, could be a diagnostic pointer. If the diagnosis is made earlier, the end stages are rarely reached as death is replaced by liver transplantation. On November 6th 1997, in Chicago, an International Faculty discussed in depth the clinical features, pathogenesis and treatment of PBC, no longer considered a rare disease. The course of PBC is long, but some 18 years after the discovery of a positive mitochondrial antibody test in a symptom­ free patient with normal serum biochemistry, 83% will have developed abnormal tests and 76% will be symptomatic. Identification of those who will progress rapidly is difficult. The serum antimitochondrial profile may be useful but this is a very specialist technique. Mathematical prognostic models are useful in therapeutic trials and in the selection and timing of patients for liver transplantation but have limited value in individual patients. An increasing serum bilirubin level remains the most important indicator of rapid progression. Its value however can be negated by the use of ursodeoxycholic acid which has a bilirubin-lowering effect.


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Editors and affiliations

  • Keith D. Lindor
    • 1
  • E. Jenny Heathcote
    • 2
  • Raoul Poupon
    • 3
  1. 1.Mayo ClinicRochesterUSA
  2. 2.The Toronto Hospital, Western DivisionUniversity of TorontoTorontoCanada
  3. 3.Service d’Hepatologie et GastroenterologieAP — Hôpital Saint-AntoineParisFrance

Bibliographic information

  • DOI
  • Copyright Information Kluwer Academic Publishers and Axcan Pharma 1998
  • Publisher Name Springer, Dordrecht
  • eBook Packages Springer Book Archive
  • Print ISBN 978-94-010-6047-9
  • Online ISBN 978-94-011-4884-9
  • Buy this book on publisher's site