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Autoimmune hepatitis

Last updated: May 15, 2020

Summary

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., type 1 diabetes mellitus, celiac disease). Clinical presentation varies and patients may be asymptomatic or even show signs of acute liver failure. Diagnosis is established based on the detection of autoantibodies (especially anti-smooth muscle antibodies) and the histologic findings of liver biopsy. Treatment consists of immunosuppressive medications such as prednisone and azathioprine. The prognosis is favorable if patients are treated appropriately; without treatment, cirrhosis may develop.

Epidemiology

References:[1]

Epidemiological data refers to the US, unless otherwise specified.

Etiology

References:[1][2][3]

Clinical features

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.

References:[1][3]

Diagnostics

Diagnosis is established based on positive autoantibodies (e.g., anti-smooth muscle antibodies) and histological findings suggestive of autoimmune hepatitis!

References:[1][2][3][4]

Differential diagnoses

The differential diagnoses listed here are not exhaustive.

Treatment

References:[5]

Prognosis

  • 10-year survival rate with treatment: ∼ 90%
    • Lifelong therapy is usually required.
    • Increased risk of developing hepatocellular carcinoma (HCC); therefore, follow-ups are recommended
  • Increased risk of liver cirrhosis if left untreated

References

  1. Wolf DC. Autoimmune Hepatitis. In: BS Anand, Autoimmune Hepatitis. New York, NY: WebMD. http://emedicine.medscape.com/article/172356. Updated: February 18, 2016. Accessed: December 27, 2016.
  2. 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
  3. Heneghan MA. Autoimmune hepatitis: Clinical manifestations and diagnosis. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/autoimmune-hepatitis-clinical-manifestations-and-diagnosis.Last updated: October 21, 2015. Accessed: December 27, 2016.
  4. Diagnosis and Management of Autoimmune Hepatitis. http://www.aasld.org/sites/default/files/guideline_documents/autoimmunehepatitis2010.pdf. Updated: February 1, 2010. Accessed: December 27, 2016.
  5. Heneghan MA. Autoimmune hepatitis: Treatment. In: Post TW, ed. UpToDate. Waltham, MA: UpToDate. https://www.uptodate.com/contents/autoimmune-hepatitis-treatment.Last updated: December 15, 2016. Accessed: December 27, 2016.
  6. Herold G. Internal Medicine. Herold G ; 2014