Abstract: Anti-neutrophil cytoplasmic antibody（ANCA）-associated vasculitis（AAV） is an autoimmune disease characterized by small-vessel inflammation，which can involve multiple organs throughout the body. The condition is extremely dangerous. With the in-depth research on pediatric AAV，more evidence has been provided for clinical guidance in the diagnosis and treatment of AAV. This article briefly elaborates on the diagnosis and treatment of pediatric AAV.

Key words: child, antineutrophil cytoplasmic antibody vasculitis, antineutrophil cytoplasmic antibody-associated nephritis, diagnosis, treatment

摘要： 抗中性粒细胞胞质抗体（ANCA）相关性血管炎（AAV）是一种以小血管炎症为特征的自身免疫性疾病，可累及全身多个器官，病情凶险。对儿童AAV研究的深入，为临床指导AAV诊治提供了更多依据，文章就儿童AAV诊断和治疗作简要阐述。

关键词: 儿童, 抗中性粒细胞胞质抗体血管炎, 抗中性粒细胞胞质抗体相关性肾炎, 诊断, 治疗