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Autoimmune hepatitis

Nature Reviews Gastroenterology & Hepatology volume 8, pages 320–329 (2011)Cite this article

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Abstract

Autoimmune hepatitis (AIH) is an inflammatory liver disease that mainly affects females. It is characterized histologically by interface hepatitis, biochemically by increased aspartate and alanine aminotransferase levels, and serologically by the presence of autoantibodies and increased levels of immunoglobulin G. AIH affects both adults and children, and is particularly aggressive in the latter group. It is a relatively rare but devastating disease, which progresses rapidly unless immunosuppressive treatment is started promptly. With appropriate treatment 80% of patients achieve remission and long-term survival. Those patients who progress to end-stage liver disease because they are unresponsive or nonadherent to treatment, and those with fulminant liver failure (encephalopathy grade II–IV) at diagnosis, require liver transplantation. Seropositivity for smooth muscle and/or antinuclear antibodies defines type 1 AIH, while positivity for liver kidney microsomal type 1 antibodies defines type 2 AIH. The primary cause of AIH is unknown; however, considerable knowledge about the mechanisms of liver damage involved has been gathered over the past 30 years, which is likely to provide the basis for specific modes of treatment and a possible cure.

Key Points

  • Autoimmune hepatitis is exquisitely responsive to corticosteroids and azathioprine treatment; if untreated, mortality at 5 years is 75%

  • Patients who do not respond to steroids, or difficult-to-treat patients, should be treated with mycophenolate mofetil and calcineurin inhibitors

  • The aim of treatment is to achieve full remission, that is, normal aspartate and alanine aminotransferase and immunoglobulin G levels and a negative or low titer of autoantibodies

  • Patients who do not achieve remission, or who relapse frequently, will need liver transplantation; risk of post-transplant recurrence is ∼20%

  • An important cause of relapse is treatment nonadherence, particularly in adolescents and young adults

  • The diagnosis for patients who do not respond to steroids should be carefully reviewed to exclude other causes of liver disease

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Figure 1: Interface hepatitis.
Figure 2: Antinuclear antibodies in autoimmune hepatitis.
Figure 3: Anti-smooth-muscle antibodies in autoimmune hepatitis.
Figure 4: Anti-liver kidney microsomal type 1 antibodies in autoimmune hepatitis.
Figure 5: Autoimmune attack to the hepatocyte.

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Both authors contributed equally to all aspects of this article.

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  1. Institute of Liver Studies, King's College London School of Medicine at King's College Hospital, Denmark Hill, SE5 9RS, London, UK

    Giorgina Mieli-Vergani & Diego Vergani

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  1. Giorgina Mieli-Vergani
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  2. Diego Vergani
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Correspondence to Giorgina Mieli-Vergani.

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Mieli-Vergani, G., Vergani, D. Autoimmune hepatitis. Nat Rev Gastroenterol Hepatol 8, 320–329 (2011). https://doi.org/10.1038/nrgastro.2011.69

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