ANCA-Associated Vasculitis: Core Curriculum 2020
ANCA-Associated Vasculitis: Core Curriculum 2020
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DOI：
10.1053/j.ajkd.2019.04.031
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发表时间：
2020-01-01
影响因子：
13.2
通讯作者：
Jefferson, J. Ashley
中科院分区：
文献类型：
--
作者：
Geetha, Duvuru;Jefferson, J. Ashley
抗中性粒细胞胞浆抗体 (ANCA) 相关性血管炎 (AAV) 是一组以中小血管炎症和破坏以及循环 ANCA 存在为特征的疾病。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，这与许多疾病特征相关。 AAV 好发于肾脏，>75% 的患者有肾脏受累，其特征是快速进展性肾小球肾炎。 AAV 的病因和发病机制是多因素的，并受到遗传、环境因素以及先天和适应性免疫系统反应的影响。过去20年的随机对照试验改进了AAV的治疗方法，并将AAV从一种致命疾病转变为一种具有复发病程和相关发病率的慢性疾病。 AJKD 肾病学核心课程系列中的这篇文章详细回顾了 AAV 的流行病学、发病机制、诊断和管理进展。
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.