Autoimmune hepatitis: new paradigms in the pathogenesis, diagnosis, and management

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Autoimmune hepatitis (AIH), primary biliary cirrhosis, and primary sclerosing cholangitis are the three major autoimmune diseases affecting the liver, and of these three, AIH is the most typical autoimmune disease being characterized by a T-cell-rich infiltrate, raised circulating gamma-globulins, autoantibodies, HLA associations, and links with other autoimmune diseases. It is the only one, of the three diseases, that responds well to immunosuppressive therapy. AIH is caused by dysregulation of immunoregulatory networks and the consequent emergence of autoreactive T cells that orchestrate a progressive destruction of hepatocytes leading untreated to liver failure. T cells play a major role in the immunopathogenesis, and both CD4(+) and CD8(+) T cells are involved together with effector responses mediated by NK cells, gamma delta T cells, and macrophages. A number of triggering factors have been proposed including viruses, xenobiotics, and drugs, but none have been conclusively shown to be involved in pathogenesis.
Original languageEnglish
Pages (from-to)475-493
Number of pages19
JournalHepatology International
Issue number2
Early online date19 May 2010
Publication statusPublished - 1 Jun 2010


  • Recruitment
  • Mycophenolate mofetil
  • Autoimmune liver disease
  • Lymphocytes
  • Th17
  • Regulatory T cell


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