Benralizumab Effectiveness in EGPA (BELONG-EGPA)
8. září 2026 aktualizováno: 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.
Přehled studie
Postavení
Nábor
Intervence / Léčba
Detailní popis
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.
Typ studie
Pozorovací
Zápis (Odhadovaný)
100
Kontakty a umístění
Tato část poskytuje kontaktní údaje pro ty, kteří studii provádějí, a informace o tom, kde se tato studie provádí.
Studijní kontakt
- Jméno: Giacomo Emmi, MD/PhD
- Telefonní číslo: +393286852815
- E-mail: giacomo.emmi0105@gmail.com
Studijní záloha kontaktů
- Jméno: Irene Mattioli, PharmD/PhD
- E-mail: irene.mattioli@unifi.it
Studijní místa
-
-
Italy
-
Florence, Italy, Itálie, 50134
- Nábor
- Edoardo Biancalana
-
Kontakt:
- Edoardo Biancalana, MD
- Telefonní číslo: +393287394992
- E-mail: edoardo.biancalana@unifi.it
-
-
Kritéria účasti
Výzkumníci hledají lidi, kteří odpovídají určitému popisu, kterému se říká kritéria způsobilosti. Některé příklady těchto kritérií jsou celkový zdravotní stav osoby nebo předchozí léčba.
Kritéria způsobilosti
Věk způsobilý ke studiu
- Dospělý
- Starší dospělý
Přijímá zdravé dobrovolníky
Ne
Metoda odběru vzorků
Vzorek nepravděpodobnosti
Studijní populace
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.
Popis
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.
Studijní plán
Tato část poskytuje podrobnosti o studijním plánu, včetně toho, jak je studie navržena a co studie měří.
Jak je studie koncipována?
Detaily designu
Kohorty a intervence
Skupina / kohorta |
Intervence / Léčba |
|---|---|
|
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.
|
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.
|
Co je měření studie?
Primární výstupní opatření
Měření výsledku |
Popis opatření |
Časové okno |
|---|---|---|
|
Complete Response
Časové okno: 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.
|
Baseline and up to 24 months (at 3, 6, 12, 24 months)
|
|
Change in Lung Function
Časové okno: 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
Časové okno: 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.
|
3, 6, 12, and 24 months
|
|
Partial response
Časové okno: 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)
|
Sekundární výstupní opatření
Měření výsledku |
Popis opatření |
Časové okno |
|---|---|---|
|
Treatment persistence
Časové okno: 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
|
|
Change in oral corticosteroid dose expressed as prednisone-equivalent dose
Časové okno: 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
|
|
Oral corticosteroid discontinuation
Časové okno: 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
Časové okno: 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
|
|
Effectiveness after benralizumab dosage switching
Časové okno: 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.
|
3 months after dosage switch
|
|
Adverse event rate
Časové okno: 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
|
Další výstupní opatření
Měření výsledku |
Popis opatření |
Časové okno |
|---|---|---|
|
Changes in organ manifestations
Časové okno: 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
Časové okno: 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
Časové okno: 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
|
|
Change in eosinophil count
Časové okno: Baseline, 3, 6, 12, and 24 months
|
Change in blood eosinophil count from baseline to each follow-up time point.
|
Baseline, 3, 6, 12, and 24 months
|
Spolupracovníci a vyšetřovatelé
Zde najdete lidi a organizace zapojené do této studie.
Sponzor
Vyšetřovatelé
- Vrchní vyšetřovatel: Giacomo Emmi, MD/PhD, University of Trieste, Italy
Publikace a užitečné odkazy
Osoba odpovědná za zadávání informací o studiu tyto publikace poskytuje dobrovolně. Mohou se týkat čehokoli, co souvisí se studiem.
Obecné publikace
- 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.
Užitečné odkazy
Termíny studijních záznamů
Tato data sledují průběh záznamů studie a předkládání souhrnných výsledků na ClinicalTrials.gov. Záznamy ze studií a hlášené výsledky jsou před zveřejněním na veřejné webové stránce přezkoumány Národní lékařskou knihovnou (NLM), aby se ujistily, že splňují specifické standardy kontroly kvality.
Hlavní termíny studia
Začátek studia (Aktuální)
11. února 2026
Primární dokončení (Odhadovaný)
11. února 2027
Dokončení studie (Odhadovaný)
11. února 2028
Termíny zápisu do studia
První předloženo
27. května 2026
První předloženo, které splnilo kritéria kontroly kvality
8. září 2026
První zveřejněno (Aktuální)
11. září 2026
Aktualizace studijních záznamů
Poslední zveřejněná aktualizace (Aktuální)
11. září 2026
Odeslaná poslední aktualizace, která splnila kritéria kontroly kvality
8. září 2026
Naposledy ověřeno
1. září 2026
Více informací
Termíny související s touto studií
Klíčová slova
Další relevantní podmínky MeSH
- Cévní onemocnění
- Kardiovaskulární choroby
- Autoimunitní onemocnění
- Onemocnění imunitního systému
- Kožní choroby
- Lymfatická onemocnění
- Lymfoproliferativní poruchy
- Kožní onemocnění, Cévní
- Vaskulitida spojená s anti-neutrofilními cytoplazmatickými protilátkami
- Granulom
- Systémová vaskulitida
- Onemocnění kůže a pojivové tkáně
- Hemická a lymfatická onemocnění
- Churg-Strauss syndrom
- Vaskulitida
- Benralizumab
Další identifikační čísla studie
- BENRA_22336_oss
- 22336_oss (Identifikátor registru: Ethics Committee of the Tuscany Region - Central Area (CEAVC))
Plán pro data jednotlivých účastníků (IPD)
Plánujete sdílet data jednotlivých účastníků (IPD)?
NE
Informace o lécích a zařízeních, studijní dokumenty
Studuje lékový produkt regulovaný americkým FDA
Ne
Studuje produkt zařízení regulovaný americkým úřadem FDA
Ne
Tyto informace byly beze změn načteny přímo z webu clinicaltrials.gov. Máte-li jakékoli požadavky na změnu, odstranění nebo aktualizaci podrobností studie, kontaktujte prosím register@clinicaltrials.gov. Jakmile bude změna implementována na clinicaltrials.gov, bude automaticky aktualizována i na našem webu .