免疫缺陷与自身炎症,免疫系统的"阴"与"阳"
Immune Deficiency and Autoinflammation,the"Yin"and"Yang"of the Immune System
周煜 1宋红梅1
作者信息
- 1. 中国医学科学院北京协和医院儿科,北京 100730
- 折叠
摘要
免疫出生缺陷(IEI)是由于基因变异导致的免疫系统疾病,通常表现为不同程度的感染、免疫失调、淋巴增殖和肿瘤倾向等.尽管最早的IEI通常是在表现为异常、反复感染的患者中诊断,但诸多研究表明,非感染表现也可能是IEI的首发甚至主要表现.在过去的10余年中,越来越多引起自身炎症的IEI被发现.尽管这些疾病很少见,但对这些疾病的研究提示免疫缺陷与自身炎症并不是截然对立的,而是作为免疫系统的阴阳两面相互影响.本文回顾了引起自身炎症的IEI涉及的机制,同时提出识别表现为自身炎症的IEI 一些线索,总结目前对于表现为自身炎症的IEI的诊治进展,并对未来IEI的相关研究提出展望.
Abstract
Inborn errors of immunity(IEI)are immune system disorders caused by genetic mutations,often presenting with varying degrees of infection,immune dysregulation,lymphoproliferation,and tumor sus-ceptibility.Initially,IEIs were typically diagnosed in patients with recurrent and unusual infections.However increasing research has shown that noninfectious manifestations can also be the initial or even primary presenta-tion of IEI.Over the past ten years,more and more IEIs associated with autoinflammatory symptoms have been identified.Although these diseases are rare,relevant research suggests that immune deficiency and autoinflam-mation are not opposing conditions but rather interconnected aspects of the immune system,influencing each other in a complementary and inseparable manner.This article reviews the mechanisms involved in IEI with au-toinflammation,and proposes some clues for identifying IEI manifested as autoinflammation.It also summarizes the current progress in the diagnosis and treatment of IEI manifested as autoinflammation,and presents pros-pects for future research on IEI.
关键词
免疫出生缺陷/自身炎症/免疫失调Key words
inborn errors of immunity/autoinflammatory/immune dysfunction引用本文复制引用
出版年
2024
NETL
NSTL
万方数据