Clinical findings on ANCA-associated renal vasculitis from the Japan RPGN registry obtained via a questionnaire survey.
Clinical findings on ANCA-associated renal vasculitis from the Japan RPGN registry obtained via a questionnaire survey.
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日本 RPGN 登记处通过问卷调查获得的 ANCA 相关肾血管炎临床结果。
DOI：
10.1007/s10157-012-0740-1
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发表时间：
2013
期刊：
影响因子：
2.3
通讯作者：
Matsuo S.
中科院分区：
文献类型：
--
作者：
Yamagata K;Usui J;Sugiyama H;Nitta K;Wada T;Muso E;Arimura Y;Koyama A;Makino H;Matsuo S.
抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）常见肾脏受累伴显著器官损害。因此，它被独立地称为ANCA相关性肾血管炎。临床上，ANCA相关性肾血管炎的特征是快速进行性肾小球肾炎。病理上，它被定义为免疫缺乏型坏死性和新月体性肾小球肾炎。根据以往来自世界各地的报道，RPGN包括ANCA相关肾血管炎的病因、患病率和预后在种族和时期之间存在差异。为了阐明日本RPGN患者的临床特征，于1999年建立了一项来自问卷调查的登记研究，并维持至2006年。因此，以前收集、分析和报告了1，772例病例。在这篇小型综述中，我们根据登记数据概述了ANCA相关肾血管炎日本患者（亚洲人）的特征性临床表现。
Renal involvement with significant organ damage is common in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). As a result, it is independently referred to ANCA-associated renal vasculitis. Clinically, ANCA-associated renal vasculitis is characterized by rapidly progressive glomerulonephritis. Pathologically, it is defined by pauci-immune type necrotizing and crescentic glomerulonephritis. According to previous reports from all over the world, the etiology, prevalence, and prognosis of RPGN including ANCA-associated renal vasculitis varies among races and periods. To elucidate the clinical characteristics of Japanese RPGN patients, a registry derived from a questionnaire survey was established in 1999 and maintained until 2006. As a result, 1,772 cases were collected, analyzed, and reported previously. In this mini-review, we outline the characteristic clinical findings of Japanese patients (Asian) with ANCA-associated renal vasculitis, based on the registry data.