不可分类的间歇性肺病和自身免疫:在儿童中面向IPAF?
Laura Aoust1, Laureline Berteloot2,3, Philippe Drabent4
1AP-HP, Hôpital Universitaire Necker-Enfants Malades, Service de Pneumologie Pédiatrique, Centre de Référence pour les Maladies Respiratoires Rares de l'Enfant, Paris, France.
Pediatric pulmonology
|September 13, 2023
概括
具有自身免疫特征的间歇性肺炎 (IPAF) 标准可以应用于患有间歇性肺病 (ILD) 和自身免疫的儿童. 这种儿科IPAF诊断显示出一个有利的过程,与连接组织疾病-ILD相比,肺纤维化较少.
科学领域:
- 儿科肺病学 儿科肺病学
- 类风湿病学 类风湿病学
- 自身免疫性疾病是一种自身免疫性疾病.
背景情况:
- 具有自身免疫特征的间歇性肺炎 (IPAF) 定义为患有间歇性肺病 (ILD) 和自身免疫的成年人.
- 根据IPAF标准,可以识别那些不符合特定结缔组织疾病 (CTD) 标准的患者.
研究的目的:
- 为了确定IPAF标准是否可以应用于儿童.
- 在儿科群体中调查IPAF的患病率,特征和临床过程.
主要方法:
- 在尼克医院 (2008-2019) 的儿科病人与ILD和自身免疫的回顾性研究.
- 根据特定的CTD和IPAF标准对儿童进行分类.
- 疾病流行病学和临床过程的分析.
主要成果:
- 在27名患者中,有6名 (4.5%) 符合IPAF标准.
- 与CTD-ILD患者相比,IPAF患者更经常是男孩 (67%对14%).
- 该过程总体上是有利的,在IPAF组与CTD-ILD (43%) 组中,对类固醇反应良好,肺纤维化较少 (0%).
结论:
- IPAF标准适用于儿童,其患病率与其他儿科ILD系列相美.
- 男孩在儿科IPAF中比在儿科CTD-ILD中更有代表性.
- 需要进行更大规模的前性研究来证实这些发现和儿科IPAF的良好预后.
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