benralizumab成功治疗嗜酸性肉芽肿病合并多血管炎对mepolizumab难治性。

IF 2.3 Multidisciplinary Respiratory Medicine Pub Date : 2021-06-24 eCollection Date: 2021-01-15 DOI:10.4081/mrm.2021.779
Francesco Menzella, Carla Galeone, Giulia Ghidoni, Patrizia Ruggiero, Silvia Capobelli, Anna Simonazzi, Chiara Catellani, Chiara Scelfo, Francesco Livrieri, Nicola Facciolongo
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引用次数: 13

摘要

简介:嗜酸性肉芽肿病合并多血管炎(EGPA)的特点是坏死性嗜酸性肉芽肿性炎症,经常累及呼吸道(90%的病例)。EGPA哮喘是系统性严重的,通常难以治疗,它是皮质类固醇耐药的,通常可以在多年前预测全身性血管炎的发作。在活化的Th-2表型中,嗜酸性粒细胞(如IL-4、IL-5和IL-13)的激活、成熟和存活所必需的细胞因子的释放发生。特别是IL-5在活性EGPA中的表达水平较高,抑制其已成为关键的治疗靶点。口服糖皮质激素(OCS)是有效的治疗选择,但不幸的是,许多患者经常复发,并导致频繁的副作用。到目前为止,还没有关于EGPA的剂量和治疗方案的官方建议。病例介绍:在这篇文章中,我们描述了一个患有EGPA,严重哮喘和慢性鼻窦炎伴鼻息肉(CRSwNP)的男性病例,尽管OCS和美polizumab治疗,哮喘和CRSwNP控制不佳。呼吸和血管炎症状在治疗切换到苯那利单抗后明显改善。在治疗期间,除了临床效果外,我们还观察到血液嗜酸性粒细胞的减少,以及肺功能测试、CT扫描和最初出现的皮肤病变的改善。结论:虽然有许多研究证实了benralizumab对EGPA的疗效,但我们报告中最有趣的方面是,在先前对mepolizumab无反应的患者中证实了疗效,而mepolizumab已知对EGPA有效。
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Successful treatment with benralizumab in a patient with eosinophilic granulomatosis with polyangiitis refractory to mepolizumab.

Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by necrotizing eosinophilic granulomatous inflammation that frequently involves the respiratory tract (90% of cases). Asthma in EGPA is systematically severe and often refractory to common treatment, it is corticosteroid resistant and can often anticipate the onset of systemic vasculitis by many years. A release of cytokines necessary for the activation, maturation and survival of eosinophils, such as IL-4, IL-5 and IL-13 occurs in the activated Th-2 phenotype. In particular, IL-5 level is high in active EGPA and its inhibition has become a key therapeutic target. Oral glucocorticoids (OCS) are effective treatment options but unfortunately, frequent relapses occur in many patients and they lead to frequent side effects. As for now, there are currently no official recommendations on doses and treatment schedules in the management of EGPA.

Case presentation: In this article, we describe the case of a man with EGPA, severe asthma and chronic rhinosinusitis with nasal polyps (CRSwNP), with poor asthma and CRSwNP control despite OCS and mepolizumab treatment. Respiratory and vasculitis symptoms improved markedly after therapeutic switch to benralizumab. During the treatment, in addition to clinical effects, we witnessed a depletion of blood eosinophils, as well as an improvement in both pulmonary function tests, CT scan and skin lesions present initially.

Conclusions: While there are many studies confirming the efficacy of benralizumab in EGPA, the most interesting aspect of our report is that efficacy was confirmed in a patient previously unresponsive to mepolizumab, known to be effective in EGPA.

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来源期刊
Multidisciplinary Respiratory Medicine
Multidisciplinary Respiratory Medicine Medicine-Pulmonary and Respiratory Medicine
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期刊介绍: Multidisciplinary Respiratory Medicine is the official journal of the Italian Respiratory Society - Società Italiana di Pneumologia (IRS/SIP). The journal publishes on all aspects of respiratory medicine and related fields, with a particular focus on interdisciplinary and translational research. The interdisciplinary nature of the journal provides a unique opportunity for researchers, clinicians and healthcare professionals across specialties to collaborate and exchange information. The journal provides a high visibility platform for the publication and dissemination of top quality original scientific articles, reviews and important position papers documenting clinical and experimental advances.
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