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Autoimmune hepatitis

Last updated: May 15, 2020


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.



Epidemiological data refers to the US, unless otherwise specified.



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.



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


Differential diagnoses

The differential diagnoses listed here are not exhaustive.




  • 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


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