Papaioannou Ourania, Sampsonas Fotios, Tsiri Panagiota, Sotiropoulou Vasilina, Christopoulos Ioannis, Komninos Dimitrios, Tzouvelekis Argyrios
Department of Respiratory Medicine, University Hospital of Patras, 26504 Patras, Greece.
Pharmaceuticals (Basel). 2025 Apr 8;18(4):543. doi: 10.3390/ph18040543.
Hypereosinophilic syndrome (HES) is a heterogeneous group of rare disorders defined by the presence of marked eosinophilia resulting in end organ damage. The diagnostic approach is multidisciplinary and treatment goals include reductions in flares and eosinophils with minimal drug-related side effects. Eleven patients ( = 11) with a diagnosis of idiopathic HES were included in the study [M/F: 6/5, median age: 54 (95% CI: 38.2 to 68.5), smokers/never smokers: 5/6]. Asthma was present in the majority of them ( = 8, 72.7%); four patients ( = 4, 36.4%) presented with eosinophilic pleural effusions, two patients ( = 2, 18.2%) with cardiac arrhythmias, and one with bilateral eyelid angioedema. Eight patients (72.7%) were treated with mepolizumab (300 mg/month) and three (27.3%) with benralizumab (30 mg/4 weeks). The median values of eosinophils at baseline and 12 months after initiation of biologic agent were 3000 (95% CI: 2172 to 11,365) K/μL and 50 (95% CI: 3 to 190) K/μL, respectively, = 0.0002. All patients with concomitant asthma ( = 8) experienced elimination of asthma flares, asthma control (ACQ < 0.75), functional improvement (mean ΔFEV1: 857 ± 594 mL), and an 82% reduction in oral corticosteroids, = 0.0001. Patients with highly characterized idiopathic HES treated with anti-eosinophilic agents between 1 October 2019 and 1 October 2023 were retrospectively included in the study. The aim of this study was to present clinical, laboratory, and functional features and outcomes in patients with thoroughly investigated idiopathic HES treated with biologic agents targeting eosinophils. Biologic agents in patients with idiopathic HES-following thorough diagnostic investigation-are both safe and effective, sparing the toxicity of immunosuppressive agents. Real-life data from larger registries are greatly anticipated.
高嗜酸性粒细胞综合征（HES）是一组罕见的异质性疾病，其定义为存在显著嗜酸性粒细胞增多并导致终末器官损害。诊断方法是多学科的，治疗目标包括减少发作次数和嗜酸性粒细胞数量，同时使药物相关副作用最小化。本研究纳入了11例诊断为特发性HES的患者[男/女：6/5，中位年龄：54岁（95%CI：38.2至68.5），吸烟者/从不吸烟者：5/6]。其中大多数患者（n = 8，72.7%）患有哮喘；4例患者（n = 4，36.4%）出现嗜酸性胸腔积液，2例患者（n = 2，18.2%）出现心律失常，1例患者出现双侧眼睑血管性水肿。8例患者（72.7%）接受了美泊利珠单抗治疗（300mg/月），3例患者（27.3%）接受了贝那利珠单抗治疗（30mg/4周）。生物制剂开始治疗前及治疗12个月时嗜酸性粒细胞的中位数值分别为3000（95%CI：2172至11365）K/μL和50（95%CI：3至190）K/μL，P = 0.0002。所有合并哮喘的患者（n = 8）哮喘发作均消除，哮喘得到控制（哮喘控制问卷评分<0.75），功能改善（平均FEV1改善值：857±594mL），口服糖皮质激素减少82%，P = 0.0001。本研究回顾性纳入了2019年10月1日至2023年10月1日期间接受抗嗜酸性粒细胞药物治疗的具有高度特征性的特发性HES患者。本研究的目的是介绍接受靶向嗜酸性粒细胞的生物制剂治疗的经过全面调查的特发性HES患者的临床、实验室和功能特征及治疗结果。对于经过全面诊断调查的特发性HES患者，生物制剂既安全又有效，避免了免疫抑制剂的毒性。非常期待来自更大规模注册研究的真实世界数据。
