[与IL-18相关的自身炎症性疾病]
Philippe Mertz1, Véronique Hentgen2, Guilaine Boursier3
1Sorbonne université, hôpital Tenon, DMU3ID, APHP, ERN RITA, Paris, France; Centre de référence des maladies auto-inflammatoires et de l'amylose inflammatoire (CEREMAIA), Paris, France; Centre hospitalier de Versailles, 78150 le Chesnay, France; Institut Cochin, Inserm, CNRS, université Paris Cité, F-75014 Paris, France.
概括
与高interleukin-18 (IL-18) 相关的自身炎症性疾病 (AIDs) 涉及天生的免疫功能障碍. 测量IL-18有助于诊断和监测这些炎症状况.
科学领域:
- 免疫学 免疫学 免疫学
- 遗传学 是一个遗传学.
- 类风湿病学 类风湿病学
背景情况:
- 自身炎症性疾病 (AID) 源于天生的免疫系统失调,导致全身炎症.
- 基因组学和理解疾病机制的进步扩大了对艾滋病的研究.
- 艾滋病的一个子集是由促进炎症的细胞因子介素-18 (IL-18) 的水平升高所定义的.
研究的目的:
- 审查有关IL-18.8功能的文献.
- 探索IL-18在艾滋病的诊断和治疗中的作用.
- 确定特定的IL-18相关的艾滋病及其临床影响.
主要方法:
- 关于IL-18和艾滋病的科学文章的文献综述.
- 分析IL-18在免疫细胞调节和炎症通路中的作用.
- 鉴定与IL-18相关的艾滋病相关的遗传突变和缺陷.
主要成果:
- IL-18对于免疫细胞的增殖,生存,分化,组织透和免疫反应的两极化至关重要.
- 与IL-18相关的艾滋病包括斯蒂尔病,NLRC4,XIAP,CDC42,PSTPIP1突变和IL-18结合蛋白缺陷.
- 这些疾病中的大多数,除了与PSTPIP1相关的疾病,风险是巨细胞激活综合征.
- 血清IL-18水平对于诊断,预后和监测这些疾病非常有价值.
结论:
- IL-18是特定类型的自身炎症性疾病的关键调解剂.
- 测量IL-18是这些疾病的宝贵诊断和监测工具.
- 准IL-18通路为艾滋病提供了潜在的治疗策略.
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