Benralizumab Effectiveness in EGPA (BELONG-EGPA)
8 settembre 2026 aggiornato da: European EGPA Study Group
Benralizumab Effectiveness in the LONG-term Real-life Setting in EGPA: The BELONG-EGPA Study
This is a multicenter, retro-prospective observational study evaluating the effectiveness and safety of benralizumab in adult patients with eosinophilic granulomatosis with polyangiitis (EGPA) in a real-world setting.
The study will include patients treated with benralizumab 30 mg every 4 weeks at EGPA referral centers participating in the European EGPA Study Group.
Clinical, laboratory, lung function, treatment, relapse, and safety data will be collected from medical charts at baseline and during follow-up up to 24 months.
Panoramica dello studio
Stato
Stato
Reclutamento
Condizioni
Condizioni
Intervento / Trattamento
Intervento / Trattamento
Descrizione dettagliata
BELONG-EGPA is a multicenter, retro-prospective observational study conducted within centers belonging to the European EGPA Study Group. The study is designed to evaluate the long-term real-world effectiveness and safety of benralizumab in adult patients with eosinophilic granulomatosis with polyangiitis (EGPA).
The study population will include adult patients with EGPA who meet the 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for EGPA or the criteria proposed in the MIRRA trial, and who receive benralizumab 30 mg every 4 weeks. Only patients with at least 3 months of available follow-up after starting benralizumab will be included.
Demographic, clinical, biological, lung function, and treatment-related data will be collected from medical charts through a standardized electronic case report form. Data will be collected at the start of benralizumab treatment and at 3, 6, 12, and 24 months of follow-up.
The primary effectiveness outcomes include achievement of complete response and partial response, changes in lung function measured by pre-bronchodilator FEV1, and disease relapses. Complete response is defined as no disease activity, with Birmingham Vasculitis Activity Score equal to 0, and an oral corticosteroid dose of 4.0 mg/day or less of prednisone, prednisolone, or equivalent. Partial response is defined as no disease activity with an oral corticosteroid dose greater than 4.0 mg/day.
Secondary outcomes include treatment persistence, oral corticosteroid tapering and discontinuation, effectiveness and safety in first-line patients compared with patients previously exposed to mepolizumab, outcomes after benralizumab dosage switching, and adverse events and serious adverse events during treatment.
Additional outcomes include changes in organ manifestations, discontinuation of disease-modifying antirheumatic drugs, changes in ANCA status, and variation in eosinophil count. All data will be anonymized and stored in a centralized database.
Tipo di studio
Tipo di studio
Osservativo
Iscrizione (Stimato)
Iscrizione
100
Contatti e Sedi
Questa sezione fornisce i recapiti di coloro che conducono lo studio e informazioni su dove viene condotto lo studio.
Contatto studio
Contatto studio
- Nome: Giacomo Emmi, MD/PhD
- Numero di telefono: +393286852815
- Email: giacomo.emmi0105@gmail.com
Backup dei contatti dello studio
- Nome: Irene Mattioli, PharmD/PhD
- Email: irene.mattioli@unifi.it
Luoghi di studio
-
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Italy
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Florence, Italy, Italia, 50134
- Reclutamento
- Edoardo Biancalana
-
Contatto:
- Edoardo Biancalana, MD
- Numero di telefono: +393287394992
- Email: edoardo.biancalana@unifi.it
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-
Criteri di partecipazione
I ricercatori cercano persone che corrispondano a una certa descrizione, chiamata criteri di ammissibilità. Alcuni esempi di questi criteri sono le condizioni generali di salute di una persona o trattamenti precedenti.
Criteri di ammissibilità
Criteri di ammissibilità
Età idonea allo studio
- Adulto
- Adulto più anziano
Accetta volontari sani
No
Metodo di campionamento
Campione non probabilistico
Popolazione di studio
Adult patients with eosinophilic granulomatosis with polyangiitis (EGPA) treated with benralizumab 30 mg every 4 weeks at EGPA referral centers participating in the European EGPA Study Group.
Patients may be newly diagnosed or have relapsing or refractory EGPA, and will be included if at least 3 months of follow-up data after benralizumab initiation are available.
Descrizione
Inclusion Criteria:
- Age 18 years or older.
- Diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA) according to the 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria or the criteria proposed in the MIRRA trial.
- Treatment with benralizumab 30 mg every 4 weeks.
- At least 3 months of available follow-up data after starting benralizumab.
- Written informed consent for study participation and data collection.
Exclusion Criteria:
- Age younger than 18 years.
- No available follow-up data after starting benralizumab.
- Follow-up after benralizumab initiation shorter than 3 months.
- Lack of written informed consent, where required by local regulations.
Piano di studio
Questa sezione fornisce i dettagli del piano di studio, compreso il modo in cui lo studio è progettato e ciò che lo studio sta misurando.
Come è strutturato lo studio?
Dettagli di progettazione
Numero di gruppi/coorti
1
Coorti e interventi
Gruppo / CoorteGruppo / Coorte |
Intervento / TrattamentoIntervento / Trattamento |
|---|---|
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Patients with EGPA treated with benralizumab
Adult patients with eosinophilic granulomatosis with polyangiitis (EGPA) receiving benralizumab 30 mg every 4 weeks in a real-world setting.
Patients may be newly diagnosed or have relapsing or refractory EGPA and must have at least 3 months of available follow-up after starting benralizumab.
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Benralizumab administered at a dose of 30 mg every 4 weeks in adult patients with eosinophilic granulomatosis with polyangiitis (EGPA) in a real-world setting.
Patients may receive benralizumab as first-line biologic therapy or after previous exposure to mepolizumab.
Treatment use, persistence, dosage changes, effectiveness outcomes, and safety events will be assessed during follow-up.
|
Cosa sta misurando lo studio?
Misure di risultato primarie
Misure di risultato primarie
Misura del risultato |
Misura Descrizione |
Lasso di tempo |
|---|---|---|
|
Complete Response
Lasso di tempo: Baseline and up to 24 months (at 3, 6, 12, 24 months)
|
Proportion of patients achieving complete response during follow-up.
Complete response is defined as no disease activity (BVAS=0) and oral corticosteroid dose ≤4.0 mg/day.
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Baseline and up to 24 months (at 3, 6, 12, 24 months)
|
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Change in Lung Function
Lasso di tempo: Baseline, 3, 6, 12, and 24 months
|
Change in pre-bronchodilator forced expiratory volume in 1 second (FEV1) from baseline to each follow-up time point. Unit of Measure: Milliliters |
Baseline, 3, 6, 12, and 24 months
|
|
Disease relapse
Lasso di tempo: 3, 6, 12, and 24 months
|
Proportion of patients experiencing disease relapse after achieving complete response.
Relapse is defined as active vasculitis, BVAS >0, and/or worsening asthma or ear-nose-throat manifestations leading to an increase in oral corticosteroid dose to >4.0 mg/day, initiation of a new immunosuppressive therapy, or hospitalization.
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3, 6, 12, and 24 months
|
|
Partial response
Lasso di tempo: Baseline and up to 24 months (at 3, 6, 12, 24 months)
|
Proportion of patients achieving complete response during follow-up.
Partial response is defined as no disease activity (BVAS=0) and oral corticosteroid dose >4.0 mg/day.
|
Baseline and up to 24 months (at 3, 6, 12, 24 months)
|
Misure di risultato secondarie
Misure di risultato secondarie
Misura del risultato |
Misura Descrizione |
Lasso di tempo |
|---|---|---|
|
Treatment persistence
Lasso di tempo: 3, 6, 12, and 24 months
|
Proportion of patients who continue benralizumab treatment, switch benralizumab dosage, or discontinue benralizumab for any reason.
|
3, 6, 12, and 24 months
|
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Change in oral corticosteroid dose expressed as prednisone-equivalent dose
Lasso di tempo: Baseline, 3, 6, 12, and 24 months
|
The daily dose of each oral corticosteroid will be converted to prednisone-equivalent mg/day using standardized glucocorticoid equivalence factors. Unit of Measure: mg/day of prednisone equivalent |
Baseline, 3, 6, 12, and 24 months
|
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Oral corticosteroid discontinuation
Lasso di tempo: 3, 6, 12, and 24 months
|
Proportion of patients who discontinue oral corticosteroid therapy during follow-up.
|
3, 6, 12, and 24 months
|
|
Overall clinical benefit in first-line versus post-mepolizumab patients
Lasso di tempo: Baseline, 3, 6, 12, and 24 months
|
A comparison of the overall clinical benefit in patients treated with benralizumab as first-line therapy versus patients switching to benralizumab following prior exposure to mepolizumab. Unit of Measure: Percentage of participants |
Baseline, 3, 6, 12, and 24 months
|
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Effectiveness after benralizumab dosage switching
Lasso di tempo: 3 months after dosage switch
|
Assessment of complete response, partial response, and changes in lung function in patients switching benralizumab dosage from 30 mg every 4 weeks to 30 mg every 8 weeks or from 30 mg every 8 weeks to 30 mg every 4 weeks.
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3 months after dosage switch
|
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Adverse event rate
Lasso di tempo: 3, 6, 12, and 24 months
|
Proportion of patients experiencing at least one adverse event during benralizumab treatment, regardless of causal association with treatment.
|
3, 6, 12, and 24 months
|
Altre misure di risultato
Altre misure di risultato
Misura del risultato |
Misura Descrizione |
Lasso di tempo |
|---|---|---|
|
Changes in organ manifestations
Lasso di tempo: Baseline, 3, 6, 12, and 24 months
|
Change in the proportion of patients with active EGPA-related organ manifestations assessed separately from Birmingham Vasculitis Activity Score items.
|
Baseline, 3, 6, 12, and 24 months
|
|
DMARD discontinuation rate
Lasso di tempo: 3, 6, 12, and 24 months
|
Proportion of patients discontinuing disease-modifying antirheumatic drug treatment that was ongoing or newly started at baseline.
|
3, 6, 12, and 24 months
|
|
Change in ANCA positivity
Lasso di tempo: Baseline, 3, 6, 12, and 24 months
|
Change in the proportion of patients with positive ANCA testing and proportion of patients with ANCA negativization among those who were ANCA-positive at baseline.
|
Baseline, 3, 6, 12, and 24 months
|
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Change in eosinophil count
Lasso di tempo: Baseline, 3, 6, 12, and 24 months
|
Change in blood eosinophil count from baseline to each follow-up time point.
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Baseline, 3, 6, 12, and 24 months
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Collaboratori e investigatori
Qui è dove troverai le persone e le organizzazioni coinvolte in questo studio.
Sponsor
Sponsor
Investigatori
Investigatori
- Investigatore principale: Giacomo Emmi, MD/PhD, University of Trieste, Italy
Pubblicazioni e link utili
La persona responsabile dell'inserimento delle informazioni sullo studio fornisce volontariamente queste pubblicazioni. Questi possono riguardare qualsiasi cosa relativa allo studio.
Pubblicazioni generali
- Hellmich B, Sanchez-Alamo B, Schirmer JH, Berti A, Blockmans D, Cid MC, Holle JU, Hollinger N, Karadag O, Kronbichler A, Little MA, Luqmani RA, Mahr A, Merkel PA, Mohammad AJ, Monti S, Mukhtyar CB, Musial J, Price-Kuehne F, Segelmark M, Teng YKO, Terrier B, Tomasson G, Vaglio A, Vassilopoulos D, Verhoeven P, Jayne D. EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update. Ann Rheum Dis. 2024 Jan 2;83(1):30-47. doi: 10.1136/ard-2022-223764.
- Wechsler ME, Akuthota P, Jayne D, Khoury P, Klion A, Langford CA, Merkel PA, Moosig F, Specks U, Cid MC, Luqmani R, Brown J, Mallett S, Philipson R, Yancey SW, Steinfeld J, Weller PF, Gleich GJ; EGPA Mepolizumab Study Team. Mepolizumab or Placebo for Eosinophilic Granulomatosis with Polyangiitis. N Engl J Med. 2017 May 18;376(20):1921-1932. doi: 10.1056/NEJMoa1702079.
- Lyons PA, Peters JE, Alberici F, Liley J, Coulson RMR, Astle W, Baldini C, Bonatti F, Cid MC, Elding H, Emmi G, Epplen J, Guillevin L, Jayne DRW, Jiang T, Gunnarsson I, Lamprecht P, Leslie S, Little MA, Martorana D, Moosig F, Neumann T, Ohlsson S, Quickert S, Ramirez GA, Rewerska B, Schett G, Sinico RA, Szczeklik W, Tesar V, Vukcevic D; European Vasculitis Genetics Consortium; Terrier B, Watts RA, Vaglio A, Holle JU, Wallace C, Smith KGC. Genome-wide association study of eosinophilic granulomatosis with polyangiitis reveals genomic loci stratified by ANCA status. Nat Commun. 2019 Nov 12;10(1):5120. doi: 10.1038/s41467-019-12515-9.
- Emmi G, Bettiol A, Gelain E, Bajema IM, Berti A, Burns S, Cid MC, Cohen Tervaert JW, Cottin V, Durante E, Holle JU, Mahr AD, Del Pero MM, Marvisi C, Mills J, Moiseev S, Moosig F, Mukhtyar C, Neumann T, Olivotto I, Salvarani C, Seeliger B, Sinico RA, Taille C, Terrier B, Venhoff N, Bertsias G, Guillevin L, Jayne DRW, Vaglio A. Evidence-Based Guideline for the diagnosis and management of eosinophilic granulomatosis with polyangiitis. Nat Rev Rheumatol. 2023 Jun;19(6):378-393. doi: 10.1038/s41584-023-00958-w. Epub 2023 May 9.
- Canzian A, Venhoff N, Urban ML, Sartorelli S, Ruppert AM, Groh M, Girszyn N, Taille C, Maurier F, Cottin V, de Moreuil C, Germain V, Samson M, Jachiet M, Denis L, Rieu V, Smets P, Pugnet G, Deroux A, Durel CA, Aouba A, Cathebras P, Deligny C, Faguer S, Gil H, Godeau B, Lifermann F, Phin-Huynh S, Ruivard M, Bonniaud P, Puechal X, Kahn JE, Thiel J, Dagna L, Guillevin L, Vaglio A, Emmi G, Terrier B; French Vasculitis Study Group and the European EGPA Study Group. Use of Biologics to Treat Relapsing and/or Refractory Eosinophilic Granulomatosis With Polyangiitis: Data From a European Collaborative Study. Arthritis Rheumatol. 2021 Mar;73(3):498-503. doi: 10.1002/art.41534. Epub 2021 Jan 23.
- Bettiol A, Urban ML, Padoan R, Groh M, Lopalco G, Egan A, Cottin V, Fraticelli P, Crimi C, Del Giacco S, Losappio L, Moi L, Cinetto F, Caminati M, Novikov P, Berti A, Cameli P, Cathebras P, Coppola A, Durel CA, Folci M, Lo Gullo A, Lombardi C, Monti S, Parronchi P, Rivera CM, Solans R, Vacca A, Espigol-Frigole G, Guarnieri G, Bianchi FC, Marchi MR, Tcherakian C, Kahn JE, Iannone F, Venerito V, Desaintjean C, Moroncini G, Nolasco S, Costanzo GAML, Schroeder JW, Ribi C, Tesi M, Gelain E, Mattioli I, Bello F, Jayne D, Prisco D, Vaglio A, Emmi G; European EGPA Study Group. Benralizumab for eosinophilic granulomatosis with polyangiitis: a retrospective, multicentre, cohort study. Lancet Rheumatol. 2023 Dec;5(12):e707-e715. doi: 10.1016/S2665-9913(23)00243-6. Epub 2023 Nov 6.
- Bettiol A, Urban ML, Dagna L, Cottin V, Franceschini F, Del Giacco S, Schiavon F, Neumann T, Lopalco G, Novikov P, Baldini C, Lombardi C, Berti A, Alberici F, Folci M, Negrini S, Sinico RA, Quartuccio L, Lunardi C, Parronchi P, Moosig F, Espigol-Frigole G, Schroeder J, Kernder AL, Monti S, Silvagni E, Crimi C, Cinetto F, Fraticelli P, Roccatello D, Vacca A, Mohammad AJ, Hellmich B, Samson M, Bargagli E, Cohen Tervaert JW, Ribi C, Fiori D, Bello F, Fagni F, Moroni L, Ramirez GA, Nasser M, Marvisi C, Toniati P, Firinu D, Padoan R, Egan A, Seeliger B, Iannone F, Salvarani C, Jayne D, Prisco D, Vaglio A, Emmi G; European EGPA Study Group. Mepolizumab for Eosinophilic Granulomatosis With Polyangiitis: A European Multicenter Observational Study. Arthritis Rheumatol. 2022 Feb;74(2):295-306. doi: 10.1002/art.41943. Epub 2021 Dec 30.
- Bettiol A, Urban ML, Bello F, Fiori D, Mattioli I, Lopalco G, Iannone F, Egan A, Dagna L, Caminati M, Negrini S, Bargagli E, Folci M, Franceschini F, Padoan R, Flossmann O, Solans R, Schroeder J, Andre M, Moi L, Parronchi P, Roccatello D, Sciascia S, Jayne D, Prisco D, Vaglio A, Emmi G; European EGPA Study Group. Sequential rituximab and mepolizumab in eosinophilic granulomatosis with polyangiitis (EGPA): a European multicentre observational study. Ann Rheum Dis. 2022 Dec;81(12):1769-1772. doi: 10.1136/ard-2022-222776. Epub 2022 Jul 18. No abstract available.
- Mattioli I, Urban ML, Padoan R, Mohammad AJ, Salvarani C, Baldini C, Berti A, Cameli P, Caminati M, Cathebras P, Bianchi FC, Cinetto F, Cohen Tervaert JW, Coppola A, Costanzo G, Cottin V, Crimi C, Del Giacco S, Desaintjean C, Egan A, Espigol-Frigole G, Folci M, Fornaro M, Franceschini F, Govoni M, Groh M, Guarnieri G, Hellmich B, Iannone F, Jones R, Kernder A, Lo Gullo A, Lombardi C, Lopalco G, Losappio L, Marchi MR, Rivera CM, Marvisi C, Maule M, Moi L, Monti S, Moosig F, Moroncini G, Negrini SM, Neumann T, Nolasco S, Novikov P, Roccatello D, Samson M, Schroeder JW, Seeliger B, Sinico RA, Solans R, Tcherakian C, Toniati P, Treppo E, Trivioli G, Vacca A, Jayne D, Bettiol A, Vaglio A, Emmi G; European EGPA Study Group. Mepolizumab versus benralizumab for eosinophilic granulomatosis with polyangiitis (EGPA): A European real-life retrospective comparative study. J Autoimmun. 2025 May;153:103398. doi: 10.1016/j.jaut.2025.103398. Epub 2025 Mar 26.
- Wechsler ME, Nair P, Terrier B, Walz B, Bourdin A, Jayne DRW, Jackson DJ, Roufosse F, Borjesson Sjo L, Fan Y, Jison M, McCrae C, Necander S, Shavit A, Walton C, Merkel PA; MANDARA Study Group. Benralizumab versus Mepolizumab for Eosinophilic Granulomatosis with Polyangiitis. N Engl J Med. 2024 Mar 7;390(10):911-921. doi: 10.1056/NEJMoa2311155. Epub 2024 Feb 23.
- Cottu A, Groh M, Desaintjean C, Marchand-Adam S, Guillevin L, Puechal X, Beaumesnil S, Lazaro E, Samson M, Taille C, Durel CA, Diot E, Nicolas S, Guilleminault L, Ebbo M, Cathebras P, Dupin C, Yildiz H, Belfeki N, Pugnet G, Chauvin P, Jouneau S, Lifermann F, Martellosio JP, Cottin V, Terrier B; French Vasculitis Study Group. Benralizumab for eosinophilic granulomatosis with polyangiitis. Ann Rheum Dis. 2023 Dec;82(12):1580-1586. doi: 10.1136/ard-2023-224624. Epub 2023 Aug 7.
- Nolasco S, Portacci A, Campisi R, Buonamico E, Pelaia C, Benfante A, Triggiani M, Spadaro G, Caiaffa MF, Scioscia G, Detoraki A, Valenti G, Papia F, Tomasello A, Crimi N, Scichilone N, Pelaia G, Carpagnano GE, Crimi C. Effectiveness and safety of anti-IL-5/Ralpha biologics in eosinophilic granulomatosis with polyangiitis: a two-year multicenter observational study. Front Immunol. 2023 Jun 30;14:1204444. doi: 10.3389/fimmu.2023.1204444. eCollection 2023.
- Guntur VP, Manka LA, Denson JL, Dunn RM, Dollin YT, Gill M, Kolakowski C, Strand MJ, Wechsler ME. Benralizumab as a Steroid-Sparing Treatment Option in Eosinophilic Granulomatosis with Polyangiitis. J Allergy Clin Immunol Pract. 2021 Mar;9(3):1186-1193.e1. doi: 10.1016/j.jaip.2020.09.054. Epub 2020 Oct 14.
- Fagni F, Bello F, Emmi G. Eosinophilic Granulomatosis With Polyangiitis: Dissecting the Pathophysiology. Front Med (Lausanne). 2021 Feb 24;8:627776. doi: 10.3389/fmed.2021.627776. eCollection 2021.
Collegamenti utili
Studiare le date dei record
Queste date tengono traccia dell'avanzamento della registrazione dello studio e dell'invio dei risultati di sintesi a ClinicalTrials.gov. I record degli studi e i risultati riportati vengono esaminati dalla National Library of Medicine (NLM) per assicurarsi che soddisfino specifici standard di controllo della qualità prima di essere pubblicati sul sito Web pubblico.
Studia le date principali
Inizio studio (Effettivo)
Inizio studio
11 febbraio 2026
Completamento primario (Stimato)
Completamento primario
11 febbraio 2027
Completamento dello studio (Stimato)
Completamento dello studio
11 febbraio 2028
Date di iscrizione allo studio
Primo inviato
Primo inviato
27 maggio 2026
Primo inviato che soddisfa i criteri di controllo qualità
Primo inviato che soddisfa i criteri di controllo qualità
8 settembre 2026
Primo Inserito (Effettivo)
Primo Inserito
11 settembre 2026
Aggiornamenti dei record di studio
Ultimo aggiornamento pubblicato (Effettivo)
Ultimo aggiornamento pubblicato
11 settembre 2026
Ultimo aggiornamento inviato che soddisfa i criteri QC
Ultimo aggiornamento inviato che soddisfa i criteri QC
8 settembre 2026
Ultimo verificato
Ultimo verificato
1 settembre 2026
Maggiori informazioni
Termini relativi a questo studio
Parole chiave
Termini MeSH pertinenti aggiuntivi
- Malattie vascolari
- Malattia cardiovascolare
- Malattie autoimmuni
- Malattie del sistema immunitario
- Malattie della pelle
- Malattie linfatiche
- Malattie linfoproliferative
- Malattie della pelle, vascolari
- Vasculite associata ad anticorpi citoplasmatici anti-neutrofili
- Granulomi
- Vasculite sistemica
- Malattie della pelle e del tessuto connettivo
- Malattie emiche e linfatiche
- Sindrome di Churg Strauss
- Vasculite
- benralizumab
Altri numeri di identificazione dello studio
Altri numeri di identificazione dello studio
- BENRA_22336_oss
- 22336_oss (Identificatore di registro: Ethics Committee of the Tuscany Region - Central Area (CEAVC))
Piano per i dati dei singoli partecipanti (IPD)
Hai intenzione di condividere i dati dei singoli partecipanti (IPD)?
NO
Informazioni su farmaci e dispositivi, documenti di studio
Studia un prodotto farmaceutico regolamentato dalla FDA degli Stati Uniti
No
Studia un dispositivo regolamentato dalla FDA degli Stati Uniti
No
Queste informazioni sono state recuperate direttamente dal sito web clinicaltrials.gov senza alcuna modifica. In caso di richieste di modifica, rimozione o aggiornamento dei dettagli dello studio, contattare register@clinicaltrials.gov. Non appena verrà implementata una modifica su clinicaltrials.gov, questa verrà aggiornata automaticamente anche sul nostro sito web .