Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) constitutes a group of rare diseases characterized by necrotizing inflammation of small blood vessels and the presence of ANCA. Increasing clinical and experimental evidences support their pathogenic role in AAV, but the exact mechanism is not fully understood. Recently, the important role of neutrophil extracellular traps (NETs) in pathogenesis of AAV is underlined. There is an indication that NETs can be a source for the formation of ANCA. The most common ANCA target antigens are myeloperoxidase (MPO) and proteinase 3 (PR3). Though the mechanism of action of ANCA is still under exploration, ANCA serology is being increasingly used for classification of AAV and revealed as kenner in defining various disease subsets associated with different genetic background, clinical features, treatment response, and prognosis. Controversy exists regarding the utility of serial measurements of ANCA in patients with AAV to monitor treatment and predict disease relapse.

中文翻译：

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抗中性粒细胞胞浆抗体 在ANCA相关血管炎的发病机制，诊断和治疗监测中的作用

抗中性粒细胞胞浆抗体（ANCA）相关血管炎（AAV）构成了一组罕见疾病，其特征在于坏死了小血管的炎症和ANCA的存在。越来越多的临床和实验证据支持它们在AAV中的致病作用，但确切的机理尚不完全清楚。最近，强调了嗜中性白细胞胞外诱捕器（NETs）在AAV发病机理中的重要作用。有迹象表明，NET可以成为ANCA形成的来源。最常见的ANCA靶抗原是髓过氧化物酶（MPO）和蛋白酶3（PR3）。尽管ANCA的作用机制仍在探索中，但ANCA血清学正越来越多地用于AAV的分类，并且在确定与不同遗传背景相关的各种疾病子集时被视为肯纳，临床特征，治疗反应和预后。关于在AAV患者中进行ANCA连续测量以监测治疗和预测疾病复发的实用性存在争议。