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Review Article| Volume 53, ISSUE 11, P1381-1393, November 2021

A reasoned approach to the treatment of autoimmune hepatitis

  • Diego Vergani
    Affiliations
    King's College London Faculty of Life Sciences & Medicine, London, UK

    Institute of Liver Studies, MowatLabs, King's College Hospital, London, UK

    Epatocentro Ticino, Lugano, Switzerland
    Search for articles by this author
  • Benedetta Terziroli Beretta-Piccoli
    Affiliations
    Institute of Liver Studies, MowatLabs, King's College Hospital, London, UK

    Epatocentro Ticino, Lugano, Switzerland

    Faculty of Biomedical Sciences, Università della Svizzera Italiana, Switzerland
    Search for articles by this author
  • Giorgina Mieli-Vergani
    Correspondence
    Corresponding author at: Emeritus Professor of Paediatric Hepatology, Paediatric Liver, GI and Nutrition Centre, MowatLabs, King's College Hospital, Denmark Hill, London SE5 9RS, UK.
    Affiliations
    King's College London Faculty of Life Sciences & Medicine, London, UK

    Epatocentro Ticino, Lugano, Switzerland

    Paediatric Liver, GI and Nutrition Centre, MowatLabs, King's College Hospital, London, UK
    Search for articles by this author

      Abstract

      Autoimmune hepatitis (AIH) is a chronic inflammatory liver disease affecting all ages, characterised by elevated transaminase and immunoglobulin G levels, positive autoantibodies, interface hepatitis on histology and good response to immunosuppressive treatment. If untreated, it has a poor prognosis. The aim of this review is to analyse AIH therapeutic interventions with reference to our knowledge of the pathogenesis of AIH. Standard treatment, based on steroids and azathioprine, leads to disease remission in 80–90% of patients. Alternative first-line treatment with budesonide is effective in adults, but less so in the juvenile form of AIH; first-line treatment with ciclosporin does not provide convincing advantages compared to standard treatment. Second-line treatments are needed for patients not responding or intolerant to first-line standard management. Mycophenolate mofetil is the most widely used second-line drug, and has good efficacy particularly for patients intolerant to azathioprine, but is teratogenic. Only few and heterogeneous data on calcineurin inhibitors and m-TOR inhibitors are available. Biologicals, including anti-tumour necrosis factor- α and anti-CD20 monoclonal antibodies, have given ambivalent results and may have severe side-effects. Clinical trials with new therapeutic options aiming at targeting B lymphocytes and proinflammatory cytokines, or expanding regulatory T cells to restore tolerance are ongoing
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