Nephrology Division, Hospital Universitari Germans Trias i Pujol, Universitat Autònoma de Barcelona, Badalona, Spain.
Nephrology Division, Hospital Universitari de Bellvitge, Barcelona, Universitat Autonoma de Barcelona, Barcelona, Spain.
Clin Rheumatol. 2018 Apr;37(4):1065-1074. doi: 10.1007/s10067-017-3973-2. Epub 2018 Mar 9.
The classification of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) remains controversial. The main objective of this study was to define the respective values of ANCA serotype-based classification, clinicopathological classification, and histopathological classification in predicting patient and renal outcomes in a Spanish cohort of patients with ANCA with specificity for myeloperoxidase, MPO-ANCA, versus ANCA with specificity for proteinase 3, PR3-ANCA. Two hundred and forty-five patients with ANCA-AAV and biopsy-proven renal involvement diagnosed between 2000 and 2104 were recruited in 12 nephrology services. Clinical and histologic data, renal outcomes, and mortality were analyzed. We applied the Chapel Hill Consensus Conference definition with categories for granulomatosis with the polyangiitis (GPA) and microscopic polyangiitis (MPA), the classification based on ANCA specificity, and the histopathological classification proposed in 2010. Eighty-two percent were MPO-ANCA positive and 18.0% PR3-ANCA positive. Altogether, 82.9% had MPA and 17.1% GPA. The median follow-up was 43.2 months (0.1-169.3). Neither ANCA-based serological nor clinical classification was predictive of renal outcomes or patient survival on bivariate or multivariate Cox regression analysis. Histopathological classification was found to predict development of end-stage renal disease (p = 0.005) in Kaplan-Meier analysis. ANCA specificity was more predictive of relapse than clinicopathological classification in multivariate analysis (HR 2.086; 95% CI 1.046-4.158; p = 0.037). In our Spanish cohort, a majority of patients had an MPO-ANCA-AAV. A classification based on ANCA specificity has a higher predictive value for relapse occurrence and could be used for decision-making with respect to induction treatment and maintenance therapies.
抗中性粒细胞胞浆抗体（ANCA）相关性血管炎（AAV）的分类仍存在争议。本研究的主要目的是确定基于抗中性粒细胞胞浆抗体（ANCA）血清型分类、临床病理分类和组织病理学分类在预测西班牙队列中髓过氧化物酶（MPO）-ANCA 与蛋白酶 3（PR3）-ANCA 特异性抗中性粒细胞胞浆抗体（ANCA）患者和肾结局方面的各自价值。在 12 个肾病服务中心招募了 245 名在 2000 年至 2014 年间诊断为 ANCA-AAV 伴肾活检证实的患者。分析了临床和组织学数据、肾结局和死亡率。我们应用 Chapel Hill 共识会议定义的分类，包括肉芽肿性多血管炎（GPA）和显微镜下多血管炎（MPA）、基于 ANCA 特异性的分类以及 2010 年提出的组织病理学分类。82%的患者为 MPO-ANCA 阳性，18.0%的患者为 PR3-ANCA 阳性。总共 82.9%的患者为 MPA，17.1%的患者为 GPA。中位随访时间为 43.2 个月（0.1-169.3）。基于 ANCA 的血清学和临床分类均不能预测肾结局或患者生存的单变量或多变量 Cox 回归分析。在 Kaplan-Meier 分析中，组织病理学分类被发现可预测终末期肾病（ESRD）的发生（p=0.005）。在多变量分析中，抗中性粒细胞胞浆抗体（ANCA）特异性比临床病理分类更能预测复发（HR 2.086；95%CI 1.046-4.158；p=0.037）。在我们的西班牙队列中，大多数患者患有 MPO-ANCA-AAV。基于 ANCA 特异性的分类对复发发生具有更高的预测价值，可用于决策诱导治疗和维持治疗。
