Abstract: The interferon-γ（IFN-γ）pathway plays an important role in hyperinflammatory response associated with pediatric rheumatic and immune diseases，particularly in macrophage activation syndrome（MAS）complicating systemic juvenile idiopathic arthritis.IFN-γ contributes to MAS progression by enhancing antigen presentation，promoting Th1 polarization，activating macrophages，and inducing chemokine release.Emapalumab，the first approved monoclonal antibody targeting IFN- γ，has been used for primary hemophagocytic lymphohistiocytosis and has shown therapeutic potential in Still’s disease-associated MAS by reducing CXCL9 levels，promoting clinical remission，and reducing glucocorticoid use.Current researches also suggest its possible therapeutic value in systemic lupus erythematosus-associated MAS，although current evidence remains limited.Attention should be paid to infection screening，vaccination management，drug interactions，and standardized off-label use in clinical application of IFN-γ.

Key words:  , interferon-γ, child, macrophage activation syndrome, emapalumab

摘要： 干扰素（IFN）-γ通路在儿童风湿免疫性疾病相关高炎症反应中具有重要作用，其中在全身型幼年特发性关节炎合并巨噬细胞活化综合征（MAS）中证据最为明确。IFN-γ可通过增强抗原递呈、促进Th1 免疫极化、活化巨噬细胞及诱导趋化因子释放，参与MAS 发生发展。依马利尤单抗作为首个获批的IFN-γ单克隆抗体，已用于治疗原发性噬血细胞性淋巴组织细胞增生症，并在Still 病相关 MAS 中显示出降低CXCL9 水平、促进缓解及减少糖皮质激素用量的潜力。现有研究还提示其在系统性红斑狼疮相关 MAS 中可能具有一定治疗价值，但证据仍有限。临床应用时需重视感染筛查、疫苗管理、药物相互作用及超说明书用药规范。

关键词: γ干扰素, 儿童, 巨噬细胞活化综合征, 依马利尤单抗