Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of disorders characterized by inflammation and destruction of small- and medium-sized blood vessels and the presence of circulating ANCA. Clinical disease phenotypes include granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis, and renal-limited vasculitis. Serologic classification of AAV into proteinase 3-ANCA disease and myeloperoxidase-ANCA disease correlates with a number of disease characteristics. AAV has a predilection for the kidney, with >75% of patients having renal involvement characterized by rapidly progressive glomerulonephritis. The cause and pathogenesis of AAV are multifactorial and influenced by genetics, environmental factors, and responses of the innate and adaptive immune system. Randomized controlled trials in the past 2 decades have refined the therapy of AAV and transformed AAV from a fatal disease to a chronic illness with relapsing course and associated morbidity. This article in AJKD's Core Curriculum in Nephrology series provides a detailed review of the epidemiology, pathogenesis, diagnosis, and advances in the management of AAV.

中文翻译：

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ANCA相关性血管炎：2020年核心课程。

抗中性粒细胞胞浆抗体（ANCA）相关血管炎（AAV）是一组疾病，其特征在于炎症和中小型血管的破坏以及循环中ANCA的存在。临床疾病表型包括肉芽肿合并多血管炎，镜下性多血管炎，嗜酸性肉芽肿合并多血管炎和肾局限性血管炎。AAV的血清学分类为蛋白酶3-ANCA疾病和髓过氧化物酶-ANCA疾病与许多疾病特征相关。AAV对肾脏有好感，> 75％的肾脏受累患者以快速进行性肾小球肾炎为特征。AAV的病因和发病机制是多因素的，并受遗传，环境因素以及先天性和适应性免疫系统反应的影响。在过去的20年中，随机对照试验完善了AAV的治疗方法，并将AAV从致命疾病转变为具有复发过程和相关发病率的慢性疾病。AJKD的“肾脏病学核心课程”系列中的这篇文章详细介绍了AAV的流行病学，发病机理，诊断和进展。