Hypereosinophilia and Hypereosinophilic Syndromes: First Findings From a Nationwide Multicenter Cohort.
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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, COHESion study group
Аннотация
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.