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Autoimmune Liver Diseases: Autoimmune Hepatitis

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Liver Disorders

Abstract

Autoimmune hepatitis is characterized by autoantibodies, hypergammaglobulinemia, and interface hepatitis. Diagnostic criteria have been codified, and diagnostic scoring systems can support clinical judgment. Prednisone or prednisolone alone or in combination with azathioprine is the standard treatment. Budesonide in combination with azathioprine can be considered in treatment-naïve individuals with mild non-cirrhotic disease or at risk for serious corticosteroid-induced complications. Treatment induces clinical, laboratory, and histological improvement in all but 7 % of patients, and the 5-year survival exceeds 90 % in most studies. Relapse occurs in 28–87 % after drug withdrawal, and treatment may be protracted. Drug withdrawal requires normalization of liver tests and liver tissue. Calcineurin inhibitors and mycophenolate mofetil have been used for corticosteroid-refractory disease or intolerance of the standard medications. Five-year survival after liver transplantation is 75–79 % in adults. Autoimmune hepatitis recurs after liver transplantation in 8–12 % after 1 year and 36–68 % after 5 years, and re-transplantation is necessary in 13–23 %.

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Acknowledgement

This review did not receive financial support from a funding agency or institution, and Albert J. Czaja, MD has no conflict of interests to declare.

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  1. Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine, 200 First Street S.W., Rochester, MN, 55905, USA

    Albert J. Czaja M.D.

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  1. Division of Gastroenterology and Hepatology, Medical College of Wisconsin , Milwaukee, Wisconsin, USA

    Kia Saeian

  2. Division of Gastroenterology and Hepatology, Medicine Medical College of Wisconsin , Milwaukee, Wisconsin, USA

    Reza Shaker

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Czaja, A.J. (2017). Autoimmune Liver Diseases: Autoimmune Hepatitis. In: Saeian, K., Shaker, R. (eds) Liver Disorders. Springer, Cham. https://doi.org/10.1007/978-3-319-30103-7_15

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