Division of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Hacettepe University, Ankara, Turkey.
Hacettepe University Vasculitis Research Centre, Ankara, Turkey.
Intern Emerg Med. 2022 Apr;17(3):743-751. doi: 10.1007/s11739-021-02863-0. Epub 2021 Oct 10.
Eosinophilic granulomatosis with polyangiitis (EGPA) is defined the disease as having two subgroups, ANCA (+) and ANCA (-). We aimed to compare EGPA subgroups in terms of clinical features, outcomes, and treatments. A multidisciplinary team was established under our vasculitis centre since October 2014. Totally 50 EGPA patients were enrolled. Clinical features, treatments, and outcomes (FFS, VDI, relapse) were reviewed. For relapse-free survival analysis, time to first relapse was compared according to ANCA phenotype by Kaplan-Meier survival analysis and log-rank test. 17 (34%) patients were in ANCA (+), 33 (66%) patients were in ANCA (-) group. ANCA (-) patients were significantly younger at the diagnosis time (37.9 ± 14.3 vs 53.8 ± 16.3; p = 0.001) and had more nasal polyposis (45.5% vs 11.8%; p = 0.017). ANCA (+) patients had higher BVAS (17[13] vs 9[4]; p = 0.002), renal involvement and peripheral neuropathy were more common in this group, while cardiac involvement was seen only in ANCA (-) group (n = 3). Biological agents (mepolizumab or rituximab) were prescribed to nine patients in ANCA (-) and two patients in ANCA (+) group. The median duration of follow-up was 47 (IQR 69.9) months. ~ 40% of patients had at least one relapse, but relapse-free survival rate was similar between the groups. However, the predictor of first relapse was elevated Ig E level [OR (95% CI): 6.5 (1.09-38.63) p = 0.04]. Consequently, both clinical features, disease activity, and treatments appear to be significantly different between EGPA subgroups. The relapse risk was similar although clinical features and treatment strategies were different. Also, elevated Ig E levels may be a precursor for the relapse.
嗜酸性肉芽肿性多血管炎（EGPA）定义为疾病有两个亚组，ANCA（+）和 ANCA（-）。我们旨在比较 EGPA 亚组的临床特征、结局和治疗。自 2014 年 10 月以来，我们的血管炎中心成立了一个多学科团队。共纳入 50 例 EGPA 患者。回顾了临床特征、治疗和结局（FFS、VDI、复发）。对于无复发生存分析，根据 ANCA 表型，通过 Kaplan-Meier 生存分析和对数秩检验比较首次复发的时间。17 例（34%）患者为 ANCA（+），33 例（66%）患者为 ANCA（-）组。ANCA（-）患者的诊断时间明显更年轻（37.9±14.3 岁 vs 53.8±16.3 岁；p=0.001），鼻息肉更多（45.5% vs 11.8%；p=0.017）。ANCA（+）患者的 BVAS 更高（17[13] vs 9[4]；p=0.002），肾脏受累和周围神经病更为常见，而心脏受累仅见于 ANCA（-）组（n=3）。九例 ANCA（-）患者和两例 ANCA（+）患者使用生物制剂（美泊利珠单抗或利妥昔单抗）治疗。中位随访时间为 47（IQR 69.9）个月。~40%的患者至少有一次复发，但两组的无复发生存率相似。然而，首次复发的预测因素是 IgE 水平升高[OR（95%CI）：6.5（1.09-38.63），p=0.04]。因此，EGPA 亚组之间的临床特征、疾病活动度和治疗均存在显著差异。尽管临床特征和治疗策略不同，但复发风险相似。此外，IgE 水平升高可能是复发的前兆。
