Autoimmune hepatitis

Last updated: August 22, 2023

Summarytoggle arrow icon

Autoimmune hepatitis (AIH) is a rare form of chronic hepatitis that predominantly affects women. Although the etiology is unclear, it is commonly associated with other autoimmune conditions (e.g., thyroid disease, type 1 diabetes mellitus, celiac disease). The clinical presentation ranges from asymptomatic transaminitis to acute liver failure. Diagnosis is established based on the detection of autoantibodies (e.g., antinuclear antibodies, anti-smooth muscle antibodies) and the histologic findings of interface hepatitis on liver biopsy. Treatment consists of immunosuppressive medications such as prednisone and azathioprine. The prognosis is favorable with treatment; without treatment, patients may develop cirrhosis and liver failure.

Classificationtoggle arrow icon

Epidemiologytoggle arrow icon

Epidemiological data refers to the US, unless otherwise specified.

Clinical featurestoggle arrow icon

AIH has an insidious onset in most patients and its presentation varies widely, ranging from asymptomatic disease to severe symptoms or even acute liver failure.

Diagnosticstoggle arrow icon

Approach [5]

  • Consider AIH in patients with unexplained liver disease.
  • Rule out other causes of hepatitis; see “Differential diagnoses.”
  • Establish the diagnosis of AIH with laboratory studies and liver histology.
  • Consider utilizing the Autoimmune Hepatitis Diagnosis calculator to facilitate the diagnosis. [6]
  • Consult hepatology for guidance on further diagnostic testing.

The diagnosis of AIH is based on positive autoantibodies (e.g., ANA, ASMA) and histological findings of interface hepatitis on biopsy.

Initial studies [5]

Laboratory tests [7][8]

Liver biopsy [7]

Additional evaluation [5]

Differential diagnosestoggle arrow icon

The differential diagnoses listed here are not exhaustive.

Treatmenttoggle arrow icon

General principles [5][7]

Pharmacotherapy [5]

Do not use azathioprine in patients with decompensated cirrhosis or acute severe AIH (i.e., jaundice, INR > 1.5). [5]

Prognosistoggle arrow icon

  • 10-year survival rate with treatment: > 90% [9]
  • Type 2 AIH is associated with more severe disease, a worse response to corticosteroids, and more frequent relapses.
  • Increased risk of liver cirrhosis if left untreated

Referencestoggle arrow icon

  1. Muri Boberg K. Prevalence and epidemiology of autoimmune hepatitis. Clin Liver Dis. 2002; 6 (3): p.635-647.doi: 10.1016/s1089-3261(02)00021-1 . | Open in Read by QxMD
  2. Baven-Pronk MAMC, Biewenga M, van Silfhout JJ, et al. Role of age in presentation, response to therapy and outcome of autoimmune hepatitis.. Clinical and translational gastroenterology. 2018; 9 (6): p.165.doi: 10.1038/s41424-018-0028-1 . | Open in Read by QxMD
  3. Czaja AJ, Donaldson PT. Gender effects and synergisms with histocompatibility leukocyte antigens in type 1 autoimmune hepatitis.. Am J Gastroenterol. 2002; 97 (8): p.2051-7.doi: 10.1111/j.1572-0241.2002.05921.x . | Open in Read by QxMD
  4. Hardtke-wolenski M, Fischer K, Noyan F, et al. Genetic predisposition and environmental danger signals initiate chronic autoimmune hepatitis driven by CD4+ T cells. Hepatology. 2013; 58 (2): p.718-28.doi: 10.1002/hep.26380 . | Open in Read by QxMD
  5. Mack CL, Adams D, Assis DN, et al. Diagnosis and Management of Autoimmune Hepatitis in Adults and Children: 2019 Practice Guidance and Guidelines From the American Association for the Study of Liver Diseases. Hepatology. 2020; 72 (2): p.671-722.doi: 10.1002/hep.31065 . | Open in Read by QxMD
  6. Alvarez F, Berg PA, Bianchi FB, et al. International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis. J Hepatol. 1999; 31 (5): p.929-938.doi: 10.1016/s0168-8278(99)80297-9 . | Open in Read by QxMD
  7. Mieli-Vergani G, Vergani D, Czaja AJ, et al. Autoimmune hepatitis. Nat Rev Dis Primers. 2018; 4 (1).doi: 10.1038/nrdp.2018.17 . | Open in Read by QxMD
  8. Kwo PY, Cohen SM, Lim JK. ACG Clinical Guideline: Evaluation of Abnormal Liver Chemistries. Am J Gastroenterol. 2017; 112 (1): p.18-35.doi: 10.1038/ajg.2016.517 . | Open in Read by QxMD
  9. Roberts S, Therneau T, Czaja A. Prognosis of histological cirrhosis in type 1 autoimmune hepatitis. Gastroenterology. 1996; 110 (3): p.848-857.doi: 10.1053/gast.1996.v110.pm8608895 . | Open in Read by QxMD

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