中国实用儿科杂志

中国实用儿科杂志 ›› 2026, Vol. 41 ›› Issue (8): 681-684.DOI: 10.19538/j.ek2026080614

• 专题笔谈 • 上一篇    下一篇

干扰素-γ单克隆抗体在儿童风湿免疫性疾病中的应用

  

  1. 电子科技大学医学院附属妇女儿童医院  成都市妇女儿童中心医院儿童风湿免疫科，四川  成都  611731
  • 出版日期:2026-08-06 发布日期:2026-09-17
  • 通讯作者: 电子信箱：gczhangwei@163.com

Application of interferon-γ monoclonal antibodies in pediatric rheumatic and immune diseases 

  1. Pediatric Immunology and Rheumatology Department，Chengdu Women and Children’s Central Hospital，School of Medicine，University of Electronic Science and Technology of China，Chengdu  611731，China
  • Online:2026-08-06 Published:2026-09-17

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

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