Abstract: Antineutrophil cytoplasmic antibody（ANCA）-associated vasculitis is a necrotizing vasculitis with few or no immune deposits，predominantly affecting small blood vessels（i.e. arterioles，venules and capillaries），including microscopic polyangiitis（MPA），granulomatosis with polyangiitis（GPA） and eosinophilic granulomatosis with polyangiitis（EPGA）. AAVs are rare diseases in children，the clinical manifestations are heterogeneous，easily to be missed and misdiagnosed；however，AAV may be organ- or life-threatening，and reasonable classification is of great significance for the diagnosis and treatment of vasculitis. The classification criteria of AAV is being constantly updated and improved due to the development of disease cognition and detection methods. This article compares different classification criteria of AAV.

Key words: ANCA, vasculitis, child, classification criteria

摘要： 抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）是一种寡或无免疫复合物沉积的坏死性血管炎，主要累及小血管（如小动脉、小静脉和毛细血管），包括显微镜下多血管炎、肉芽肿性多血管炎和嗜酸性肉芽肿性多血管炎。儿童AAV罕见，临床表现异质性，极易被漏诊和误诊，但可危及器官和生命，合理分类对于血管炎诊断和治疗具有重要意义。由于对疾病的认知和检测手段的发展，AAV的分类标准也在不断更新和完善，文章就不同AAV分类标准进行比较。

关键词: 中性粒细胞胞浆抗体, 血管炎, 儿童, 分类标准