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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Article info
Publication history
Published online: June 21, 2021
Accepted:
May 27,
2021
Received:
February 26,
2021
Identification
Copyright
© 2021 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.