Kwon Oh Chan, Ha Jang Woo, Park Min-Chan, Park Yong-Beom, Lee Sang-Won
Division of Rheumatology, Department of Internal Medicine, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul 06273, Republic of Korea.
Division of Rheumatology, Department of Internal Medicine, Yongin Severance Hospital, Yonsei University College of Medicine, Yongin 16995, Republic of Korea.
Medicina (Kaunas). 2025 Jun 13;61(6):1088. doi: 10.3390/medicina61061088.
: Previous studies have suggested differences in vasculitic and eosinophilic phenotypes based on anti-neutrophil cytoplasmic antibody (ANCA) positivity in eosinophilic granulomatosis with polyangiitis (EGPA). However, their relevance under the 2022 American College of Rheumatology (ACR)/European Alliance of Associations for Rheumatology (EULAR) classification criteria remains unclear. We aimed to evaluate the clinical features and outcomes of EGPA according to myeloperoxidase (MPO)-ANCA status in a Korean cohort. : We conducted a retrospective cohort study that included 57 patients with EGPA without proteinase 3-ANCA positivity who fulfilled the 2022 ACR/EULAR classification criteria. Patients were classified into MPO-ANCA-positive (n = 25) and MPO-ANCA-negative (n = 32) groups. Clinical manifestations, laboratory findings, and outcomes, including all-cause mortality, relapse, end-stage kidney disease (ESKD), cerebrovascular accident (CVA), and acute coronary syndrome (ACS), were compared between the two groups. : MPO-ANCA-positive patients exhibited higher Five-Factor Scores (1.0 [0.0-1.0] vs. 0.0 [0.0-1.0], = 0.038), lower Short Form 36 Physical Component Summary scores (35.0 [19.7-56.3] vs. 52.5 [43.5-69.7], = 0.048), and elevated systemic inflammation markers (higher erythrocyte sedimentation rate: 58.0 [16.0-97.5] mm/hr vs. 25.5 [7.0-63.8] mm/hr, = 0.026). Constitutional symptoms were more frequent among MPO-ANCA-positive patients (n = 14 [56.0%] vs. n = 3 [9.4%], < 0.001), whereas no significant differences were found in vasculitic or eosinophilic manifestations. Kaplan-Meier analysis revealed no differences in the overall ( = 0.36), relapse-free ( = 0.80), ESKD-free ( = 0.87), CVA-free ( = 0.26), or ACS-free ( = 0.94) survival rates between the two groups. : In Korean patients with EGPA classified under the 2022 ACR/EULAR classification criteria, MPO-ANCA positivity, as compared to ANCA-negative status, was associated with a higher disease burden and poorer quality of life but not with distinct vasculitic or eosinophilic manifestations and adverse outcomes.
以往研究表明，在嗜酸性肉芽肿性多血管炎（EGPA）中，基于抗中性粒细胞胞浆抗体（ANCA）阳性情况，血管炎和嗜酸性粒细胞表型存在差异。然而，在2022年美国风湿病学会（ACR）/欧洲抗风湿病联盟（EULAR）分类标准下，它们的相关性仍不明确。我们旨在评估韩国队列中根据髓过氧化物酶（MPO）-ANCA状态的EGPA临床特征和结局。
我们进行了一项回顾性队列研究，纳入了57例符合2022年ACR/EULAR分类标准且无蛋白酶3-ANCA阳性的EGPA患者。患者被分为MPO-ANCA阳性组（n = 25）和MPO-ANCA阴性组（n = 32）。比较了两组的临床表现、实验室检查结果和结局，包括全因死亡率、复发、终末期肾病（ESKD）、脑血管意外（CVA）和急性冠状动脉综合征（ACS）。
MPO-ANCA阳性患者表现出更高的五因素评分（1.0 [0.0 - 1.0] 对 0.0 [0.0 - 1.0]，P = 0.038）、更低的简明健康状况调查36项身体成分汇总评分（35.0 [19.7 - 56.3] 对 52.5 [43.5 - 69.7]，P = 0.048）以及升高的全身炎症标志物（更高的红细胞沉降率：58.0 [16.0 - 97.5] mm/小时 对 25.5 [7.0 - 63.8] mm/小时，P = 0.026）。MPO-ANCA阳性患者的全身症状更常见（n = 14 [56.0%] 对 n = 3 [9.4%]，P < 0.001），而在血管炎或嗜酸性粒细胞表现方面未发现显著差异。Kaplan-Meier分析显示两组在总体生存率（P = 0.36）、无复发生存率（P = 0.80）、无ESKD生存率（P = 0.87）、无CVA生存率（P = 0.26）或无ACS生存率（P = 0.94）方面无差异。
在根据2022年ACR/EULAR分类标准分类的韩国EGPA患者中，与ANCA阴性状态相比，MPO-ANCA阳性与更高的疾病负担和更差的生活质量相关，但与独特的血管炎或嗜酸性粒细胞表现及不良结局无关。
