在全身性青少年异常性关节炎中,先天性淋巴细胞的表型和功能变化
Linda Quatrini1, Cecilia Ciancaglini2, Ivan Caiello3
1Innate Lymphoid Cells Unit, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
Arthritis & rheumatology (Hoboken, N.J.)
|May 13, 2025
概括
缓解期的系统性青春性异常性关节炎 (sJIA) 患者仍然表现出先天性免疫细胞异常. 天生的淋巴细胞 (ILC) 和介素-18 (IL-18) 水平发生变化,表明sJIA中正在进行的免疫失调.
科学领域:
- 免疫学 免疫学 免疫学
- 儿科风湿病学 儿科风湿病学
背景情况:
- 系统性青少年异常性关节炎 (SJIA) 是一种慢性儿童自发炎症疾病.
- 天生的免疫细胞失调是sJIA的一个标志.
- 在临床不活跃疾病 (CID) 期间,先天性淋巴细胞 (ILCs) 在sJIA中的作用仍然不清楚.
研究的目的:
- 调查ILCs的表型和功能特征,包括自然杀手细胞 (NK) 和辅助ILCs (hILCs),在CID期间患有sJIA的儿童中.
- 在sJIA中探索ILCs和细胞因子配置,特别是IL-18 (IL-18) 之间的关联.
主要方法:
- 流细胞测量分析外周血液ILCs从sJIA患者的CID (n=40) 在IL-1抑制剂,与健康儿童 (HC,n=23) 和其他自身炎症性疾病的患者相比.
- 血蛋白质组分析以评估细胞因子水平.
- 功能性试验评估NK细胞和hILC细胞因子的产生 (IFN-γ,IL-13).
主要成果:
- 与HC相比,CID中的sJIA患者表现出降低的NK细胞频率,CD56明亮NK细胞的比例较高.
- 在sJIA患者中观察到ILC1s的增加和ILC前体 (ILCPs) 的减少.
- 观察到NK细胞和hILCs的IFN-γ产生受损,与血IL-18水平升高相反相关,可能与IL-18受体α (IL-18RA) 在ILC1s表达减少有关.
结论:
- 在CID中患有sJIA的儿童表现出显著的先天性免疫异常,包括改变ILC子集分布和受损的IFN-γ生产.
- 这些免疫变化与升高的IL-18水平密切相关,这表明正在进行的免疫失调.
- ILCs及其与IL-18等细胞因子的相互作用可能在sJIA的发病过程中发挥关键作用,即使在临床缓解期间.
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