抗γ-干扰素自身抗体介导成人起病型免疫缺陷综合征研究进展

doi:10.3969/j.issn.1673-8640.2026.07.017

摘要/Abstract

摘要：

成人起病型免疫缺陷综合征（AOID）是一种罕见的免疫系统疾病，其特点在于患者体内产生针对γ-干扰素（IFN-γ）的中和性抗γ-干扰素自身抗体（nAIGA）。nAIGA会中和IFN-γ，使干扰素信号传导发生障碍，进而影响单核-巨噬细胞中信号转导和转录激活因子1（STAT1）的磷酸化和白细胞介素-12（IL-12）的分泌，导致严重的辅助性T细胞（Th）1免疫反应缺陷。AOID患者常出现多种机会性病原体感染，如分枝杆菌、马尔尼菲篮状菌、曲霉和沙门菌属感染。因抗γ-干扰素自身抗体综合征多发生于既往健康的成人，所以，根据其发病机制和临床特征命名为成人起病型免疫缺陷综合征（AOID）。目前，AOID的发病机制尚不明确，诊断和治疗均尚未标准化。文章全面阐述了nAICA介导的AOID的发病机制、临床特点、检测方法、诊断和治疗研究进展，以期能提高临床对AOID的认识，为制定标准诊疗方案提供参考。

关键词: 中和性抗干扰素-γ自身抗体, 成人起病型免疫缺陷综合征, 机会性病原体

Abstract:

Adult-onset immunodeficiency syndrome（AOID） is a rare immune system disorder characterized by the production of neutralizing anti-interferon-gamma（IFN-γ） autoantibodies（nAIGA） in patients' bodies. These nAIGA can neutralize IFN-γ，causing impairment in interferon signal transduction and affecting the phosphorylation of signal transducer and activator of transcription 1（STAT1） and the secretion of interleukin-12（IL-12） in mononuclear-macrophages，resulting in severe T helper cell（Th）1 immune response deficiency. These AOID patients often suffer from various opportunistic pathogen infections，such as Mycobacterium，Malphigian basidiomycetes，Aspergillus and Salmonella infections. Since anti-interferon-gamma autoantibody syndrome mostly occurs in previously healthy adults，it is named AOID based on its pathogenesis and clinical characteristics. Currently，the pathogenesis，diagnosis and treatment of AOID are not yet clear and standardized. This review comprehensively elaborates on the research progress in the pathogenesis，clinical characteristics，determination methods，diagnosis and treatment of nAIDA-mediated AOID，with the aim of enhancing clinicians' understanding of AOID，and to provide a reference for the formulation of standard treatment plans.

Key words: Neutralizing anti-interferon-gamma autoantibody, Adult-onset immunodeficiency syndrome, Opportunistic pathogen

中图分类号:

R446.62

刘耀婷, 钟驾云, 周琳. 抗γ-干扰素自身抗体介导成人起病型免疫缺陷综合征研究进展[J]. 检验医学, 2026, 41(7): 714-718.

LIU Yaoting, ZHONG Jiayun, ZHOU Lin. Research progress on anti-interferon-gamma autoantibodies mediating adult-onset immunodeficiency syndrome[J]. Laboratory Medicine, 2026, 41(7): 714-718.

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