a Vasculitis Clinic, Division of Rheumatology , Mount Sinai Hospital, University Health Network , Toronto , Canada.
b Division of Rheumatology, Mount Sinai Hospital, University Health Network , Toronto Allergy and Asthma Clinic , Toronto , Canada.
Expert Opin Biol Ther. 2019 Jul;19(7):617-630. doi: 10.1080/14712598.2019.1623875. Epub 2019 May 31.
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis characterized by asthma, hypereosinophilia, and progressive multiorgan involvement. Although not fully elucidated, advancement in our understanding of the pathophysiology of EGPA has led to the development of multiple new treatment targets.
Herein we review the epidemiology, clinical manifestations, pathophysiology, treatments, and ongoing research in the management of EGPA. The central role of Interleukin-5 (IL-5) in the development and maintenance of hypereosinophilia will be discussed. The value of mepolizumab, an anti-IL-5 monoclonal antibody, in the treatment of EGPA is reviewed in detail.
The available literature supports the use of mepolizumab for the induction and maintenance of remission of refractory, relapsing, or glucocorticoid-dependent EGPA with potentially greater benefit in those who are ANCA-positive or those with greater eosinophilia ( 150 cells/ L). Despite these positive results, relapses remain frequent, and the need for both short- and long-term glucocorticoid use remains common. More research is needed to address these needs and determine the precise role of mepolizumab.
嗜酸性肉芽肿伴多血管炎（EGPA）是一种罕见的抗中性粒细胞胞浆抗体（ANCA）相关性血管炎，其特征是哮喘、嗜酸性粒细胞增多和进行性多器官受累。尽管尚未完全阐明，但我们对 EGPA 病理生理学的理解的提高导致了多个新的治疗靶点的发展。
本文综述了 EGPA 的流行病学、临床表现、病理生理学、治疗方法以及管理方面的正在进行的研究。将讨论白细胞介素 5（IL-5）在嗜酸性粒细胞增多症的发展和维持中的核心作用。详细回顾了抗 IL-5 单克隆抗体美泊利珠单抗在 EGPA 治疗中的应用价值。
现有文献支持使用美泊利珠单抗诱导和维持难治性、复发性或依赖糖皮质激素的 EGPA 缓解，对于 ANCA 阳性或嗜酸性粒细胞计数较高（ 150 个/ L）的患者可能获益更大。尽管取得了这些积极的结果，但仍经常复发，并且仍然需要短期和长期使用糖皮质激素。需要进一步研究以解决这些需求并确定美泊利珠单抗的确切作用。
