ANCA‐associated vasculitis (AAV) is a group of chronic inflammatory diseases of small‐ and medium‐sized vessels, which are broadly subdivided based on organ manifestations and disease‐specific autoantibodies. The so called anti‐neutrophil cytoplasmic antibodies (ANCA) mostly target one of the enzymes, proteinase 3 (PR3) or myeloperoxidase (MPO). Accumulating genetic data demonstrates that these two autoantibodies discriminate two distinct disease entities, more so than the clinical subdivision which is mainly criteria‐based. Treatment of AAV includes heavy immunosuppression and is guided by which organs that are involved. Generally, patients with PR3‐ANCA display higher risk for disease relapse than patients with MPO‐ANCA. In this review, we will focus on the autoimmune features of PR3+ AAV and our current understanding of its triggers and the potential translation into clinical practice.

中文翻译：

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抗中性粒细胞胞质抗体相关性血管炎中的蛋白酶 3 自身反应性 - 免疫学与临床特征。

ANCA相关性血管炎（AAV）是一组中小血管的慢性炎症性疾病，根据器官表现和疾病特异性自身抗体可大致细分。所谓的抗中性粒细胞胞浆抗体 (ANCA) 主要针对其中一种酶：蛋白酶 3 (PR3) 或髓过氧化物酶 (MPO)。积累的遗传数据表明，这两种自身抗体能够区分两种不同的疾病实体，比主要基于标准的临床细分更能区分。AAV 的治疗包括严重的免疫抑制，并以受影响的器官为指导。一般来说，PR3-ANCA 患者的疾病复发风险高于 MPO-ANCA 患者。在这篇综述中，我们将重点关注 PR3+ AAV 的自身免疫特征以及我们目前对其触发因素的理解以及转化为临床实践的潜力。