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美泊利单抗在真实临床实践中治疗嗜酸性肉芽肿性多血管炎患者的疗效和安全性:来自西班牙风湿病科回顾性多中心登记的数据。

Mepolizumab efficacy and safety in patients with eosinophilic granulomatosis with polyangiitis in real practice: Data from a Spanish retrospective multicentric register from Rheumatology departments.

作者信息

Rubiño Juárez Francisco, García de Vicuña Rosario, Vicente Esther, Rodríguez Almaraz Esther, Lozano Rivas Nuria, Freire González Mercedes, Olivé Marqués Alejandro, Nack Annika, Narváez García Javier, Moya Alvarado Patricia, Uriarte Itzazelaia Esther, Alcorta Lorenzo Nerea, Marenco de la Fuente José Luis, Ramos Consuelo, Pinillos Aransay Valvanera, Blanco Alonso Ricardo, Benavides Villanueva Fabricio, Martínez Vidal María Paz, González Martín Jesús María, Rúa-Figueroa Íñigo

机构信息

University Hospital of Gran Canaria Doctor Negrín, Las Palmas de Gran Canaria, Spain.

University Hospital La Princesa, Madrid, Spain.

出版信息

Reumatol Clin (Engl Ed). 2025 Jan;21(1):101806. doi: 10.1016/j.reumae.2025.101806.

Abstract

OBJECTIVES

Eosinophilic granulomatosis with polyangiitis (EGPA) is a type of ANCA-associated vasculitis mostly mediated by eosinophils. Mepolizumab (MPZ) reduces the absolute number of eosinophils in the peripheral blood and tissues and has proven efficacy in the maintenance of EGPA, as shown in a randomized controlled trial. The aim of this study is to describe the use of MPZ in EGPA in real clinical practice.

METHODS

This is a descriptive, retrospective and comparative analysis of the clinical features, course, response rates, outcomes and adverse effects of patients receiving MPZ for EGPA in thirteen Spanish Rheumatology departments.

RESULTS

A total of 30 EGPA patients treated with MPZ were included in the analysis. Up to 19 patients needed at least one concomitant immunosuppressant. The mean follow-up was 16 months. The use of MPZ reduced the median Birmingham Vasculitis Activity Score (BVAS), as well as the mean C-reactive protein and the levels of eosinophils. In addition, the median dose of glucocorticoids (GC) required was reduced in 79.3% of the patients and was completely suspended in 57.1%. Interestingly, the median Vasculitis Damage Index (VDI) was also calculated in 27 patients (90%), and remained stable (3 [0-12] pre-MPZ and 3 [0-12] post-MPZ). None of the patients suffered severe adverse effect, local reactions or serious infections during the treatment.

CONCLUSIONS

Our data on the real practice use of MPZ in EGPA suggests that this drug has good efficacy, is safe and reduces the dependence on glucocorticoids or even their complete withdrawal. Furthermore, it appears to prevent the organic damage progression associated with this disease.

摘要

目的

嗜酸性肉芽肿性多血管炎(EGPA)是一种主要由嗜酸性粒细胞介导的抗中性粒细胞胞浆抗体相关性血管炎。美泊利单抗(MPZ)可减少外周血和组织中嗜酸性粒细胞的绝对数量,并且如一项随机对照试验所示,已证实其在维持EGPA病情缓解方面具有疗效。本研究的目的是描述MPZ在实际临床实践中治疗EGPA的应用情况。

方法

这是一项对西班牙13个风湿病科接受MPZ治疗EGPA的患者的临床特征、病程、缓解率、转归及不良反应进行的描述性、回顾性和对比分析。

结果

共有30例接受MPZ治疗的EGPA患者纳入分析。多达19例患者需要至少一种联合免疫抑制剂。平均随访时间为16个月。使用MPZ可降低伯明翰血管炎活动评分(BVAS)中位数,以及C反应蛋白均值和嗜酸性粒细胞水平。此外,79.3%的患者所需糖皮质激素(GC)的剂量中位数降低,57.1%的患者GC完全停用。有趣的是,还对27例患者(90%)计算了血管炎损伤指数(VDI)中位数,且其保持稳定(MPZ治疗前为3[0 - 12],MPZ治疗后为3[0 - 12])。治疗期间无患者出现严重不良反应、局部反应或严重感染。

结论

我们关于MPZ在EGPA实际临床应用的数据表明,该药物疗效良好、安全,可减少对糖皮质激素的依赖甚至完全停用糖皮质激素。此外,它似乎可预防与该疾病相关的器质性损伤进展。

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