Department of Pulmonology, Clinical Hospital Dubrava, School of Medicine, University of Zagreb, Zagreb, Croatia.
J Asthma. 2021 Dec;58(12):1675-1679. doi: 10.1080/02770903.2020.1827417. Epub 2020 Oct 1.
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of small-vessel vasculitis characterized by asthma, hyper-eosinophilia, and progressive multiorgan involvement. EGPA is traditionally treated using glucocorticoids, either alone or in combination with conventional immunosuppressants. Mepolizumab, a novel anti-interleukin (IL)-5 agent, has been approved as an add-on therapy for adult patients with EGPA. The recommended dose of mepolizumab is 300 mg (subcutaneous [SC]) every 4 weeks.
The present report discusses three cases of refractory and/or relapsing EGPA in patients regularly taking a stable dose of prednisolone, all of whom were successfully treated with a lower-than-recommended dose of mepolizumab (100 mg SC, every 4 weeks).
Treatment with a low dose of mepolizumab enabled us to gradually reduce glucocorticoid doses. In addition, patients treated with low doses of mepolizumab exhibited better asthma control and experienced sustained relapse-free periods. Responses to 100 mg of mepolizumab were comparable to those previously observed in patients taking the recommended dose of 300 mg.
Our findings suggest that mepolizumab at a third of the recommended dose can achieve reasonable clinical efficacy in the long-term treatment of EGPA in some patients. Initiation of mepolizumab therapy in the early, eosinophilic phase of EGPA-which is characterized by asthma, marked blood eosinophilia, pulmonary infiltrates, and sinonasal abnormalities-may help to prevent the deleterious side-effects of long-term glucocorticoid use while reducing the cost of EGPA treatment.
嗜酸性肉芽肿性多血管炎（EGPA）是一种罕见的小血管血管炎，其特征是哮喘、嗜酸性粒细胞增多和进行性多器官受累。EGPA 的传统治疗方法是使用糖皮质激素，单独使用或与传统免疫抑制剂联合使用。美泊利珠单抗是一种新型抗白细胞介素（IL）-5 药物，已被批准用于治疗成人 EGPA 患者的附加治疗。美泊利珠单抗的推荐剂量为 300mg（皮下[SC]），每 4 周一次。
本报告讨论了三例常规服用稳定剂量泼尼松龙的难治性和/或复发性 EGPA 患者，他们均成功接受了低于推荐剂量的美泊利珠单抗（100mg SC，每 4 周一次）治疗。
使用低剂量美泊利珠单抗治疗使我们能够逐渐减少糖皮质激素剂量。此外，接受低剂量美泊利珠单抗治疗的患者哮喘控制更好，且无复发期持续时间更长。对 100mg 美泊利珠单抗的应答与之前观察到的接受推荐剂量 300mg 美泊利珠单抗的患者相当。
我们的研究结果表明，对于某些患者，美泊利珠单抗的推荐剂量的三分之一可在 EGPA 的长期治疗中获得合理的临床疗效。在 EGPA 的嗜酸性粒细胞期（以哮喘、显著血嗜酸性粒细胞增多、肺部浸润和鼻窦异常为特征）早期开始美泊利珠单抗治疗可能有助于预防长期使用糖皮质激素的不良副作用，同时降低 EGPA 治疗的成本。
