JLE

Hépato-Gastro & Oncologie Digestive

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Autoimmune hepatitis Volume 24, issue 8, Octobre 2017

Author
CHU de Nantes, Hôtel Dieu, Institut des Maladies de l’Appareil Digestif (IMAD), 1 place Alexis Ricordeau, 44093 Nantes cedex 01, France
* Tirés à part

Autoimmune hepatitis (AIH) was described by Waldenström in the 1950s. The first case was a young woman with chronic liver disease associated with jaundice, amenorrhea and hypergammaglobulinemia. In the 1970s, three studies demonstrated the efficacy of corticosteroid therapy alone or in combination with azathioprine: normalization of liver and immunological tests, improvement of liver histological lesions and increase in overall survival. In 1995, one study confirmed the efficacy of azathioprine as a maintenance therapy for AIH. In 2010, a controlled randomized trial demonstrated the superiority of budesonide on prednisone, both in combination with azathioprine in the treatment of patients with AIH without cirrhosis.

Studies have provided a better understanding of the immune response in AIH. It has been shown that there is an interaction between immunity, genetic factors, and exogenous factors such as viral infections or certain drugs. New autoantibodies have been identified (anti-SLA, anti-LC1, anti-lactoferrin antibodies). They are useful for diagnosing AIH in atypical forms or in the absence of conventional antibodies. However, we are still awaiting the identification of new therapeutic targets.

Diagnostic scores, including the 2008 simplified score, can help in diagnosis, which is sometimes difficult. The place of non-invasive techniques for evaluating fibrosis is still debated. For 20 years, the overlap syndrome associating AIH with primary biliary cirrhosis or AIH and primary sclerosing cholangitis have been widely described. Diagnostic criteria for these mixed forms have been established

New therapies have been studied in AIH, particularly in intolerant patients or patients resistant to the standard treatment with corticosteroids and azathioprine. Mycophenolate mofetil is an alternative to azathioprine in intolerant patients. Cyclosporine or tacrolimus is the second treatment line to be used in patients who fail standard therapy. Rituximab, and may also be infliximab, can be used as a life-saving treatment in severe patients with therapeutic failure.