第二线免疫疗法在新发症耐药状态发症患者中
Aurélie Hanin1,2,3, Eyal Muscal4, Lawrence J Hirsch1
1Comprehensive Epilepsy Center, Department of Neurology, Yale University School of Medicine, New Haven, Connecticut, USA.
像阿纳金拉和托西利祖马布这样的二线免疫疗法对控制新发性耐火状态 (NORSE) 和发烧性感染相关综合征 (FIRES) 的发作充满希望. 早期细胞因子测量可以指导治疗,但长期结果需要进一步研究.
科学领域:
- 神经免疫学 神经免疫学
- 发病学 (Epileptology) 是一个专业的学科.
- 临床药理学 临床药理学
背景情况:
- 免疫失调与新发性耐火状态 (NORSE) 和其亚型,发烧性感染相关综合征 (FIRES) 的病原发生有关.
- 已建立的指导方针建议护士/火焰患者使用一线和二线免疫疗法.
研究的目的:
- 审查有关NORSE/FIRES二线免疫疗法的疗效和结果的当前文献.
- 分析在NORSE患者队列中使用anakinra,tocilizumab和内甲的使用情况.
主要方法:
- 对28个案例报告和系列的系统审查.
- 分析了75名患者的数据,这些患者接受了阿纳金拉,托西利祖马布或内甲治疗.
- 检查治疗开始时间,控制,细胞因子水平和长期结果.
主要成果:
- 在很大一部分患者中,阿纳金拉 (73%),托西利祖马布 (70%) 和内甲 (50%) 在两周内控制了发作.
- 治疗后细胞因子水平普遍下降;在儿科FIRES中,阿纳金拉和内甲更常见,而在成人中,托西利祖马布更常见.
- 大多数患者经历了长期残疾和耐药性;在某些情况下,免疫治疗的晚期启动显示出潜在的发作减少.
结论:
- 阿纳金拉,托西利祖马布和内德克萨米他显示出作为NORSE/FIRES的二线治疗的潜在实用性.
- 早期的细胞因子水平监测可能有助于治疗选择和反应评估.
- 进一步的前性研究对于阐明早期免疫调节在和功能结果中的作用至关重要.
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