23. Autoimmune Hepatitis and Overlap Syndromes

  1. James S. Dooley MD, FRCP2,3,
  2. Anna S. F. Lok MBBS, MD, FRCP4,
  3. Andrew K. Burroughs FRCP, FMedSci3,5 and
  4. E. Jenny Heathcote MB BS, MD, FRCP, FRCP(C)6,7
  1. Gideon M. Hirschfield MBBChir, MRCP, PhD1 and
  2. E. Jenny Heathcote MB BS, MD, FRCP, FRCP(C)6,7

Published Online: 5 MAY 2011

DOI: 10.1002/9781444341294.ch23

Sherlock's Diseases of the Liver and Biliary System, 12th Edition

Sherlock's Diseases of the Liver and Biliary System, 12th Edition

How to Cite

Hirschfield, G. M. and Heathcote, E. J. (2011) Autoimmune Hepatitis and Overlap Syndromes, in Sherlock's Diseases of the Liver and Biliary System, 12th Edition (eds J. S. Dooley, A. S. F. Lok, A. K. Burroughs and E. J. Heathcote), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781444341294.ch23

Editor Information

  1. 2

    Centre for Hepatology, University College London Medical School, UK

  2. 3

    Royal Free Sheila Sherlock Liver Centre, Royal Free Hospital, London, UK

  3. 4

    Division of Gastroenterology, University of Michigan Health System, Ann Arbor, MI, USA

  4. 5

    University College London, London, UK

  5. 6

    Division of Gastroenterology, University Health Network, University of Toronto, Toronto, Ontario, Canada

  6. 7

    Patient Based Clinical Research, Toronto Western Hospital Research Institute, Toronto, Ontario, Canada

Author Information

  1. 1

    Liver Centre, Toronto Western Hospital, Toronto, ON, Canada

  2. 6

    Division of Gastroenterology, University Health Network, University of Toronto, Toronto, Ontario, Canada

  3. 7

    Patient Based Clinical Research, Toronto Western Hospital Research Institute, Toronto, Ontario, Canada

Publication History

  1. Published Online: 5 MAY 2011
  2. Published Print: 25 APR 2011

ISBN Information

Print ISBN: 9781405134897

Online ISBN: 9781444341294

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Keywords:

  • primary biliary cirrhosis;
  • primary sclerosing cholangitis;
  • autoantibodies;
  • transaminitis;
  • hypergammaglobulinaemia

Summary

Autoimmune hepatitis is a chronic relapsing and remitting hepatitis, seen across all ages and in both genders. Disease severity ranges from mild asymptomatic inflammation to acute liver failure. The syndrome of autoimmune hepatitis lacks a definitive diagnostic test and disease is characterized broadly by transaminitis, hypergammaglobulinaemia and the presence of autoantibodies. Histology whilst not unique in its own right includes a plasma cell rich interface hepatitis. Exclusion of drug-induced liver injury and Wilson's disease is important. Therapy with prednisone and azathioprine is successful in the majority of cases in terms of inducing disease remission and preventing disease relapse. Some patients may have outlier features of other autoimmune liver diseases, including primary biliary cirrhosis and primary sclerosing cholangitis. Overlap syndromes are, however, not distinct diseases.