Abstract: Antineutrophil Cytoplasmic Antibody associated vasculitis （AAV） is a group of small necrotizing vasculitis with little or no deposition of immune complex. It is rare in children and can involve multiple organs and is life-threatening. Kidney involvement is common and can result in ANCA-associated glomerulonephritis （AAGN）， which has a high probability of progression to end-stage renal disease and is a key factor determining the prognosis of the disease. In view of the rarity of AAV in children， there are few randomized controlled trials and guidelines for children， and most of them refer to recommendations from  adult guidelines， especially for the diagnosis and treatment of AAGN.This article aims to briefly review the research progress in the diagnosis and treatment of AAGN in recent years.

Key words: child, ANCA-associated vasculitis, ANCA-asso-ciated nephritis, ANCA-associated vasculitis renal injury, treatment, necrotizing crescentic nephritis

摘要： 抗中性粒细胞胞浆抗体（antineutrophil cytoplas-mic antibody，ANCA）相关性血管炎（antineutrophil cytoplas-mic antibody associated vasculitis，AAV）是一组寡或无免疫复合物沉积的坏死性小血管炎，儿童罕见，可累及多个器官，危及生命。肾脏受累常见，可导致ANCA相关性肾小球肾炎（ANCA-associated glomerulonephritis，AAGN），其进展至终末期肾病的概率较大，是决定该疾病预后的关键因素。而鉴于儿童罕见的特点，关于该疾病的随机对照试验研究及针对儿童的循证指南少，特别是针对儿童AAGN的诊治多借鉴成人指南的建议，文章旨在就近年来关于AAGN诊治方面的研究进展作简要综述。

关键词: 儿童, ANCA相关性血管炎, ANCA相关性肾炎, ANCA相关性血管炎肾损伤, 治疗, 坏死性新月体性肾炎