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