Konstantouli Afroditi Maria, Lioulios Georgios, Stai Stamatia, Moysidou Eleni, Fylaktou Asimina, Papagianni Aikaterini, Stangou Maria
Department of Nephrology, Hippokration General Hospital, Aristotle University of Thessaloniki, 54642 Thessaloniki, Greece.
Department of Immunology, National Histocompatibility Center, Hippokration General Hospital, 54642 Thessaloniki, Greece.
Life (Basel). 2022 Sep 21;12(10):1467. doi: 10.3390/life12101467.
The traditional nomenclature system for classifying antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) based on clinical phenotype describes granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA) and microscopic polyangiitis (MPA) as distinct clinical entities. This classification has proved its expedience in clinical trials and everyday clinical practice; yet, a substantial overlap in clinical presentation still exists and often causes difficulties in prompt definition and clinical distinction. Additionally, new insights into the AAV pathogenesis point out that PR3 and MPO-AAV may not represent expressions of the same disease spectrum but rather two distinct disorders, as they display significant differences. Thus, it is supported that a classification based on ANCA serotype (PR3-ANCA, MPO-ANCA or ANCA-negative) could be more accurate and also closer to the nature of the disease compared to the phenotype-based one. This review aims to elucidate the major differences between PR3 and MPO-AAV in terms of epidemiology, pathogenesis, histological and clinical manifestations and response to therapeutic approaches.
基于临床表型对抗中性粒细胞胞浆抗体（ANCA）相关血管炎（AAV）进行分类的传统命名系统将肉芽肿性多血管炎（GPA）、嗜酸性肉芽肿性多血管炎（EGPA）和显微镜下多血管炎（MPA）描述为不同的临床实体。这种分类在临床试验和日常临床实践中已证明其便利性；然而，临床表现仍存在大量重叠，常常导致在快速定义和临床鉴别方面出现困难。此外，对AAV发病机制的新见解指出，PR3-AAV和MPO-AAV可能并非同一疾病谱的表现，而是两种不同的疾病，因为它们存在显著差异。因此，有人支持基于ANCA血清型（PR3-ANCA、MPO-ANCA或ANCA阴性）的分类可能比基于表型的分类更准确，也更接近疾病本质。本综述旨在阐明PR3-AAV和MPO-AAV在流行病学、发病机制、组织学和临床表现以及对治疗方法的反应方面的主要差异。
