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Review
. 2021 Oct:72:318-323.
doi: 10.1016/j.coi.2021.08.001. Epub 2021 Aug 26.

Candidiasis in patients with APS-1: low IL-17, high IFN-γ, or both?

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Review

Candidiasis in patients with APS-1: low IL-17, high IFN-γ, or both?

Quentin Philippot et al. Curr Opin Immunol. 2021 Oct.

Abstract

Chronic mucocutaneous candidiasis (CMC) is one of the earliest and most frequent clinical manifestations of autosomal recessive autoimmune polyendocrine syndrome type 1 (APS-1), a monogenic inborn error of immunity caused by deleterious variants of the autoimmune regulator (AIRE) gene. APS-1 patients suffer from various autoimmune diseases, due to the defective thymic deletion of autoreactive T cells, and the development of a large range of autoantibodies (auto-Abs) against various tissue antigens, and some cytokines. The mechanisms underlying CMC remained elusive for many years, until the description in 2010 of high serum titers of neutralizing auto-Abs against IL-17A, IL-17F, and/or IL-22, which are present in almost all APS-1 patients. Excessively high mucosal concentrations of IFN-γ were recently proposed as an alternative mechanism for CMC in APS-1.

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