高嗜酸性粒细胞增多症和高嗜酸性粒细胞综合征：来自全国多中心队列的初步发现
Hypereosinophilia and Hypereosinophilic Syndromes: First Findings From a Nationwide Multicenter Cohort.
作者信息
Lefèvre Guillaume, Bleuse Séverine, Puyade Mathieu, Moulis Guillaume, Néel Antoine, Abisror Noémie, Baudet Antoine, Bonnotte Bernard, Dion Jérémie, Dossier Antoine, Grall Maximilien, Lifermann François, Limal Nicolas, Lioger Bertrand, Machelart Irène, Mohr Catherine, Outh Roderau, Queyrel-Moranne Viviane, Slama Borhane, Tréfond Ludovic, Abou Chahla Wadih, Ackerman Félix, Belfeki Nabil, Berezne Alice, Blade Jean-Sébastien, Bouderbala Mohammed Azzedine, Chebrek Safia, Cottin Vincent, De Almeida Sébastien, De Masson Adèle, Dezoteux Frédéric, Goulenok Tiphaine, Jachiet Vincent, Jouvray Mathieu, Latu Irina, Ledoult Emmanuel, Leurs Amélie, Lugosi Maxime, Martin Michael, Melboucy-Belkhir Sara, Morati-Hafsaoui Chafika, Quemeneur Thomas, Rohmer Julien, Roy-Peaud Frédérique, Sanges Sébastien, Schleinitz Nicolas, Staumont-Salle Delphine, Taillé Camille, Terriou Louis, Tieulie Nathalie, Koenga Japhete Darline Elenga, Schwarb Laurent, Panel Kewin, Kahn Jean-Emmanuel, Groh Matthieu
机构信息
Institut d'Immunologie, Service de médecine Interne et d'immunologie Clinique, CHU Lille, INFINITE-Institute for Translational Research in Inflammation, Université de Lille, Lille, France.
Centre de Référence des Syndromes Hyperéosinophiliques (CEREO), Suresnes, France.
出版信息
Allergy. 2025 Apr;80(4):1100-1110. doi: 10.1111/all.16463. Epub 2025 Jan 5.
BACKGROUND
Hypereosinophilic syndromes (HES) are a heterogenous group of eosinophilic disorders. To date, only retrospective studies of limited sample-size and/or follow-up duration are available.
METHODS
The COHESion study is a national prospective multicenter multidisciplinary cohort recruiting both adults or children with the spectrum of eosinophilic disorders (including reactive HE/HES [HE/HES-R], idiopathic HES [HES-I], lymphocytic HES [HES-L], neoplastic HE/HES [HE/HES-N], HE of unknown significance [HE-US], as well as IgG4-related disease [IgG4RD] or ANCA-negative eosinophilic granulomatosis with polyangiitis [EGPA] overlaps). Patients are followed-up yearly. All data about final diagnosis, organ involvement assessments, and outcome profiles in HES-I were captured and analyzed centrally by HES expert centers.
RESULTS
From May 2019 to November 2023, 779 patients were included. For this preliminary analysis, 550 cases were available for centralized review (mean ± SD age: 56 ± 18 years, 42% of female patients). The final diagnoses were HES-I (47%), HE/HES-R (16%), HE-US (15%), HE/HES-N (7%), HE/HES-L (6%), IgG4RD (2%), and ANCA-negative EGPA (7%). In the 258 HES-I patients, outcome profiles were classified as follows: 16.3% had a "single-flare" without further relapse, 28.3% had a "relapsing-remitting" disease when there was at least a 6-month period free of symptoms between two flares, 46.1% had a "persistent disease" requiring continuous treatment to avoid relapses (9.3% remained unclassified because of insufficient follow-up).
CONCLUSIONS
The COHESion cohort is the first nationwide prospective multicenter study collecting data on the full spectrum of HE/HES disorders. This preliminary analysis confirms that idiopathic HES patients have various outcome profiles, suggesting different underlying pathophysiological mechanisms and the need of patient-specific management.
TRIAL REGISTRATION
ClinicalTrials.gov identifier: NCT04018118.
背景
高嗜酸性粒细胞综合征（HES）是一组异质性嗜酸性粒细胞疾病。迄今为止，仅有样本量和/或随访时间有限的回顾性研究。
方法
COHESion研究是一项全国性前瞻性多中心多学科队列研究，招募患有嗜酸性粒细胞疾病谱的成人或儿童（包括反应性HE/HES [HE/HES-R]、特发性HES [HES-I]、淋巴细胞性HES [HES-L]、肿瘤性HE/HES [HE/HES-N]、意义未明的HE [HE-US]，以及IgG4相关疾病[IgG4RD]或抗中性粒细胞胞浆抗体阴性的嗜酸性肉芽肿性多血管炎[EGPA]重叠情况）。患者每年接受随访。HES专家中心集中收集并分析所有关于HES-I的最终诊断、器官受累评估和结局情况的数据。
结果
2019年5月至2023年11月，共纳入779例患者。对于本次初步分析，有550例病例可供集中审查（平均年龄±标准差：56±18岁，女性患者占42%）。最终诊断为HES-I（47%）、HE/HES-R（16%）、HE-US（15%）、HE/HES-N（7%）、HE/HES-L（6%）、IgG4RD（2%）和抗中性粒细胞胞浆抗体阴性的EGPA（7%）。在258例HES-I患者中，结局情况分类如下：16.3%为“单次发作”且无进一步复发，28.3%为“复发缓解型”疾病，即两次发作之间至少有6个月无症状期，46.1%为“持续性疾病”，需要持续治疗以避免复发（9.3%因随访不足未分类）。
结论
COHESion队列是第一项收集关于整个HE/HES疾病谱数据的全国性前瞻性多中心研究。本次初步分析证实，特发性HES患者有多种结局情况，提示存在不同的潜在病理生理机制以及需要针对患者个体进行管理。
试验注册
ClinicalTrials.gov标识符：NCT04018118。
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