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儿童开始的ANCA相关血管炎:来自中加州的单一中心经验

Deepika Singh¹, Sukesh Sukumaran²

¹Division of Pediatric Rheumatology, Department of Pediatrics, Valley Children's Healthcare, Madera, CA, USA. DSingh2@valleychildrens.org.

Pediatric rheumatology online journal

|July 3, 2023

概括

微观多炎 (MPA) 是中加州最常见的儿童ANCA相关血管炎 (AAV),影响更多的女性和少数民族儿童. 在MPA患者中,最初的疾病表现更为严重.

科学领域:

儿科风湿病学儿科风湿病学
免疫学免疫学免疫学
血管炎研究血管炎研究

背景情况:

儿童发病的ANCA相关的血管类型 (AAV) 包括多炎的粒状瘤 (GPA),微观多炎 (MPA) 和多炎的eosinophilic粒状瘤 (EGPA).
关于儿科AAV特征的数据有限,特别是在加利福尼亚中部.

研究的目的:

为了检查中央加利福尼亚州儿科AAV患者的特征.
分析童年开始的AAV的初始表现,治疗和结果.

主要方法:

在2010年至2021年间诊断的18岁或更年轻的AAV患者的回顾性研究.
对人口统计,临床,实验室数据,治疗和初始结果的分析.

主要成果:

微观多叶膜炎 (MPA) 的发生率比多叶膜炎 (GPA) 的颗粒瘤病更高 (12/21名患者) (9/21).
在MPA队列中,女性占优势 (92%),种族/少数民族占比较高 (67%是西班牙裔),症状持续时间较短 (14天).
在MPA患者中,ICU入院率 (67%) 和透析率 (50%) 较高,报告有两例死亡. 在GPA患者中,经常出现耳鼻关 (89%). 所有患者都是ANCA阳性;西班牙裔患者是MPO阳性,而白人患者是PR3阳性.

结论:

微观多管炎是这一儿科队列中占主导地位的AAV亚型,具有明显的人口和临床特征.
患有AAV的西班牙裔儿童经常显示MPO阳性. MPA病例呈现出更严重的初始疾病.
需要进一步的前性研究,以阐明儿童时期发病的AAV在不同种族和民族群体的表现和结果的差异.

关键词:

与ANCA相关的血管炎带有多炎的埃索诺菲尔颗粒瘤症.微观多炎,与多炎一起发生的粒状炎.小血管血管炎是一种血管炎.血管炎是一种血管炎.

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