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Observational Study
. 2025 Apr;80(4):1100-1110.
doi: 10.1111/all.16463. Epub 2025 Jan 5.

Hypereosinophilia and Hypereosinophilic Syndromes: First Findings From a Nationwide Multicenter Cohort

Guillaume Lefèvre  1   2 , Séverine Bleuse  1   2 , Mathieu Puyade  3 , Guillaume Moulis  4 , Antoine Néel  5 , Noémie Abisror  6 , Antoine Baudet  7 , Bernard Bonnotte  8 , Jérémie Dion  9 , Antoine Dossier  10 , Maximilien Grall  11 , François Lifermann  12 , Nicolas Limal  13 , Bertrand Lioger  14 , Irène Machelart  15 , Catherine Mohr  16 , Roderau Outh  17 , Viviane Queyrel-Moranne  18 , Borhane Slama  19 , Ludovic Tréfond  20 , Wadih Abou Chahla  2   21 , Félix Ackerman  2   22 , Nabil Belfeki  23 , Alice Berezne  7 , Jean-Sébastien Blade  24 , Mohammed Azzedine Bouderbala  25 , Safia Chebrek  19 , Vincent Cottin  26 , Sébastien De Almeida  27 , Adèle De Masson  28 , Frédéric Dezoteux  2   29 , Tiphaine Goulenok  10 , Vincent Jachiet  6 , Mathieu Jouvray  30 , Irina Latu  31 , Emmanuel Ledoult  2   32 , Amélie Leurs  33 , Maxime Lugosi  34 , Michael Martin  3 , Sara Melboucy-Belkhir  35 , Chafika Morati-Hafsaoui  7 , Thomas Quemeneur  36 , Julien Rohmer  2   22 , Frédérique Roy-Peaud  3 , Sébastien Sanges  2   32 , Nicolas Schleinitz  37 , Delphine Staumont-Salle  2   29 , Camille Taillé  38 , Louis Terriou  2   32 , Nathalie Tieulie  18 , Japhete Darline Elenga Koenga  2   39 , Laurent Schwarb  39 , Kewin Panel  2 , Jean-Emmanuel Kahn  2   40 , Matthieu Groh  2   22 ; COHESion study group
Collaborators, Affiliations
Observational Study

Hypereosinophilia and Hypereosinophilic Syndromes: First Findings From a Nationwide Multicenter Cohort

Guillaume Lefèvre et al. Allergy. 2025 Apr.

Abstract

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.

Keywords: eosinophil; eosinophilic granulomatosis with polyangiitis; hypereosinophilia; hypereosinophilic syndrome.

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References

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