Abstract
Autoimmune hepatitis (AIH) can present as acute hepatitis in its onset of symptoms and features, progressing to severe hepatitis and acute liver failure. In a recent national survey in Japan, 10.9 % of the patients with AIH were diagnosed with acute hepatitis. Some patients with acute presentation of AIH lack the typical features of AIH, such as increased serum immunoglobulin G levels and high autoantibody titers. For accurate diagnosis, it is essential to exclude the other possible causes that can lead to acute hepatitis, and liver biopsy should be performed early if appropriate. Centrilobular necrosis without interface hepatitis or fibrosis is considered to be the histological characteristic in the acute hepatitis phase of AIH. Interface hepatitis is a hallmark characteristic of AIH; therefore, its absence in acute hepatitis phase can lead to missed diagnosis of AIH. Most patients with an acute presentation of AIH respond well to corticosteroid therapy; however, the prognosis of AIH patients worsens with progression to acute liver failure. Liver transplantation is a possible treatment option for patients with acute liver failure who do not respond to corticosteroid therapy.
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Acknowledgements
This study was supported by a Grant-in-Aid from the Intractable Hepatobiliary Diseases Study Group of Japan, supported by the Japanese Ministry of Health, Labor and Welfare.
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Abe, M., Onji, M. (2014). Acute Presentation of Autoimmune Hepatitis. In: Ohira, H. (eds) Autoimmune Liver Diseases. Springer, Tokyo. https://doi.org/10.1007/978-4-431-54789-1_6
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DOI: https://doi.org/10.1007/978-4-431-54789-1_6
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