Antineutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) is a term used to describe rare primary systemic vasculitides affecting small and medium-sized blood vessels. AAV diseases which include Granulomatosis with Polyangiitis (GPA), Eosinophilic Granulomatosis with Polyangiitis (EGPA), Microscopic Polyangiitis (MPA) and renal limited ANCA vasculitis. These multisystemic disorders involve upper and lower respiratory tract and kidneys associated with organ damage and long term sequelae. Newer understanding of pathogenesis in AAV have paved the way for clinical research with different biologic therapies. In spite of the paucity of clinical trials in pediatric AAV, the long-term survival of patients with AAV has improved dramatically. International collaborations will help to conduct clinical trials in pediatric AAV and help in better understanding of remission rates, relapse rates, and other outcomes. This article aims to provide a comprehensive review of pediatric AAV with a focus on epidemiology, disease pathogenesis, treatment trials, and prognosis.

中文翻译：

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儿童GPA，EGPA和MPA。

抗中性粒细胞胞浆抗体（ANCA）相关血管炎（AAV）是一个术语，用于描述影响中小型血管的罕见原发性系统性血管炎。AAV疾病包括肉芽肿性多血管炎（GPA），嗜酸性肉芽肿性多血管炎（EGPA），镜下性多血管炎（MPA）和肾脏局限性ANCA血管炎。这些多系统疾病涉及上，下呼吸道以及与器官损害和长期后遗症相关的肾脏。对AAV发病机理的新认识为采用不同生物疗法的临床研究铺平了道路。尽管缺乏针对儿科AAV的临床试验，但AAV患者的长期存活率仍得到了显着提高。国际合作将有助于开展儿科AAV的临床试验，并有助于更好地了解缓解率，复发率和其他结局。本文旨在提供对儿科AAV的全面综述，重点是流行病学，疾病发病机理，治疗试验和预后。