Division of Nephrology, Johns Hopkins University, Baltimore, MD.
Division of Nephrology, University of Washington, Seattle, WA.
Am J Kidney Dis. 2020 Jan;75(1):124-137. doi: 10.1053/j.ajkd.2019.04.031. Epub 2019 Jul 26.
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.
抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）是一组以小血管和中等大小血管炎症和破坏为特征，并伴有循环抗中性粒细胞胞浆抗体（ANCA）的疾病。临床疾病表型包括肉芽肿性多血管炎、显微镜下多血管炎、嗜酸性肉芽肿性多血管炎和肾血管炎。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 偏爱肾脏，>75%的患者有肾脏受累的特征，表现为快速进行性肾小球肾炎。AAV 的病因和发病机制是多因素的，受遗传、环境因素以及先天和适应性免疫系统反应的影响。过去 20 年的随机对照试验对 AAV 的治疗进行了优化，并将 AAV 从一种致命性疾病转变为一种具有复发性和相关发病率的慢性疾病。本文是 AJKD 的肾脏病学核心课程系列中的一篇，详细回顾了 AAV 的流行病学、发病机制、诊断和治疗进展。
