极度周期性发烧,口腔口炎,耳炎,腺炎 (PFAPA):一个独立的患者群体
Mor Broide1, Yoel Levinsky2,3, Rotem Tal2,3
1Department of Pediatrics A, Schneider Children's Medical Center of Israel, Petach Tikva, Israel. morbrd@gmail.com.
Pediatric rheumatology online journal
|September 1, 2023
概括
极端PFAPA综合征 (周期性发烧,口腔口炎,喉炎和宫腺炎) 在儿童中比典型PFAPA更早地出现. 大约一半的极端病例从一开始就表现出症状,而其他人则在接受皮质类固醇治疗后出现症状.
科学领域:
- 儿科 儿科 儿科
- 免疫学 免疫学 免疫学
- 类风湿病学 类风湿病学
背景情况:
- 周期性发烧,口腔炎,喉炎和宫腺炎 (PFAPA) 综合征是儿童常见的自身炎症性疾病.
- 典型的PFAPA发作每隔2到8周就会发生一次.
- 一部分患者出现更频繁的发作 (<2周),称为极端PFAPA (ePFAPA).
研究的目的:
- 描述ePFAPA的人口和临床特征.
- 将ePFAPA患者与非极端PFAPA (nPFAPA) 患者进行比较.
- 分析ePFAPA亚组:表现极端的人与在皮质类固醇治疗后出现极端症状的人.
主要方法:
- 365名PFAPA患者的医疗记录的回顾性审查 (2014年3月至2021年4月).
- 排除同时患有家族地中海热病的患者.
- 使用威尔科克森等级总和,皮尔森的千平方和费舍尔的精确测试,对ePFAPA和nPFAPA组进行统计比较.
主要成果:
- 有47名患者 (12.9%) 被发现患有ePFAPA.
- 与nPFAPA患者相比,ePFAPA患者的疾病发病年龄较早 (中位数为1.5岁对2.5岁),诊断年龄较早 (中位数为2.6岁对4.5岁).
- 较高比例的ePFAPA患者接受了胆固醇预防药物 (53%对19%),在爆发症状上没有显著差异.
结论:
- 大约一半的ePFAPA患者最初出现了极端症状.
- 与nPFAPA相比,ePFAPA与较早的发病和诊断有关.
- 两个ePFAPA子组的人口和临床特征相似.
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