Related Experiment Video
Updated: Jul 8, 2025
Induction of Drug-Induced, Autoimmune Hepatitis in BALB/c Mice for the Study of Its Pathogenic Mechanisms
Published on: May 29, 2020
Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy-Associated Hepatitis
Zhubene Mesbah1,2, Nishant Tiwari3, Keith Sacco3
1Carl T. Hayden Veterans' Administration Medical Center, Phoenix, AZ.
Abstract:
Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is an inborn error of immunity, resulting from variation in the autoimmune regulator gene (AIRE). Pathogenic variants in the AIRE gene result in autoimmunity typically involving endocrine organs with nonendocrine organs less commonly affected. Hepatitis associated with APECED has emerged as a potentially fatal complication with higher reported prevalence in the Americas. We describe a case of a 3-year-old boy presenting with hepatitis from APECED without classical clinical diagnostic criteria. This case highlights the importance of APECED in the evaluation of hepatitis given response to immunomodulator treatment and risk of fulminate liver failure.
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