没有区分的自身炎症性疾病:系统性审查
Si-Ming Peng1,2, Zhong-Xun Yu1,2, Hong-Mei Song3,4
1Department of Pediatrics, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, No. 1 Shuaifuyuan, Dongcheng District, Beijing 100730, China.
World journal of pediatrics : WJP
|December 5, 2025
概括
不分化的自身炎症性疾病 (UAID) 影响了21%的自身炎症性疾病患者. 发烧,关节痛和腹痛是常见的,IL-1生物药物显示高缓解率.
科学领域:
- 类风湿病学 类风湿病学
- 免疫学 免疫学 免疫学
- 遗传学 遗传学 是一个
背景情况:
- 不分化的自身炎症性疾病 (UAID) 是一种独特的类别的自身炎症性疾病 (AID).
- uAIDs包括那些不符合已确定的单基或多基AIDs诊断标准的患者.
- 本综述阐明了UAIDs的患病率,临床特征和治疗结果.
研究的目的:
- 为了确定UAIDs的综合流行率.
- 要总结UAIDs的临床表现.
- 审查被诊断患有UAIDs的患者的治疗反应.
主要方法:
- 按照PRISMA指南,对主要数据库 (PubMed,科学网,EMBASE,Cochrane,Scopus) 的系统文献审查.
- 包括英语研究,根据临床表现定义uAIDs,排除其他AIDs,以及负面/不确定的遗传测试.
- 进行元分析,以计算UAIDs的综合流行率.
主要成果:
- 分析了35篇文章,通过系统性炎症定义uAIDs,缺乏其他AIDs的标准,以及负面的基因测试.
- 在艾滋病患者中,UAID的综合患病率为21% (95% CI:14-29).
- 发烧,关节痛和腹痛是最常见的症状;针对IL-1的生物药物实现了70%的缓解.
结论:
- 在患有自身炎症特征且对已确定的诊断没有反应的患者中,应考虑使用无抗炎药物.
- IL-1通路与uAIDs有关,治疗反应证明了这一点.
- 标准化诊断标准和前性研究对于推动UAID研究至关重要.
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