22. Autoimmune Hepatitis

  1. Eugene R. Schiff MD, MACP, FRCP2,
  2. Willis C. Maddrey MD, MACP, FRCP3 and
  3. Michael F. Sorrell MD, FACP4
  1. Edward L. Krawitt MD

Published Online: 31 OCT 2011

DOI: 10.1002/9781119950509.ch22

Schiff's Diseases of the Liver, Eleventh Edition

Schiff's Diseases of the Liver, Eleventh Edition

How to Cite

Krawitt, E. L. (2011) Autoimmune Hepatitis, 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.ch22

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. University of Vermont College of Medicine, Burlington, VT, USA

Publication History

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

ISBN Information

Print ISBN: 9780470654682

Online ISBN: 9781119950509

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Keywords:

  • Autoantibody;
  • autoantibodies;
  • autoimmunity;
  • autoimmune hepatitis;
  • autoimmune liver disease;
  • cholangitis;
  • chronic hepatitis;
  • cirrhosis;
  • γ-globulin;
  • HLA;
  • immune regulation;
  • immune suppression;
  • primary biliary cirrhosis;
  • primary sclerosing cholangitis

Summary

Autoimmune hepatitis is a chronic hepatitis occurring worldwide in children and adults that can progress to cirrhosis. It is thought to occur as a result of escape from the normal suppression of self-reactivity. One paradigm for its pathogenesis embraces the concept that in a genetically predisposed individual, an environmental agent triggers an autoimmune pathogenic process affecting the liver. The predominant genetic associations are with the human leukocyte antigen region of the major histocompatibilty locus on chromosome 6. Viruses, immunizations, herbs, and drugs have been proposed as triggering agents. The clinical manifestations are highly variable and sometimes follow a fluctuating course. On occasion, variant or overlap syndromes occur with primary biliary cirrhosis or primary sclerosing cholangitis. Diagnosis is based on histologic, clinical, biochemical, and serologic findings. Circulating autoantibodies, an elevated serum immunoglobulin G, and an abnormal liver biochemical profile in the presence of histologically confirmed chronic hepatitis, in the absence of viral or drug-induced disease, support the diagnosis. Immunosuppressive treatment generally induces remission, but long-term maintenance therapy is often required. In patients unresponsive to or intolerant of medical therapy, liver transplantation may be required.