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Candidiasis in patients with APS-1: low IL-17, high IFN-γ, or both?
Current opinion in immunology Pub Date : 2021-08-26 , DOI: 10.1016/j.coi.2021.08.001
Quentin Philippot 1 , Jean-Laurent Casanova 2 , Anne Puel 3
Affiliation  

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.

中文翻译:

APS-1 患者的念珠菌病:低 IL-17,高 IFN-γ,或两者兼而有之?

慢性皮肤黏膜念珠菌病 (CMC) 是 1 型常染色体隐性自身免疫性多内分泌综合征 (APS-1) 最早和最常见的临床表现之一,这是一种由自身免疫调节因子 (AIRE) 基因的有害变异引起的单基因先天免疫错误。由于自身反应性 T 细胞的胸腺缺失缺陷,以及针对各种组织抗原和一些细胞因子的大量自身抗体 (auto-Abs) 的发展,APS-1 患者患有各种自身免疫性疾病。多年来,CMC 的潜在机制一直难以捉摸,直到 2010 年描述了针对 IL-17A、IL-17F 和/或 IL-22 的高血清中和自身抗体滴度,这些抗体几乎存在于所有 APS-1 患者中.
更新日期:2021-08-26
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