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Glucocorticoids versus glucocorticoids plus cyclophosphamide in eosinophilic granulomatosis with polyangiitis with poor-prognosis factors

2024-12Journal article Restricted access
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dc.contributor.authorSorin, Boris
dc.contributor.authorPapo, Matthias
dc.contributor.authorSinico, Renato A.
dc.contributor.authorTeixeira, Vítor Silvestre
dc.contributor.authorVenhoff, Nils
dc.contributor.authorUrban, Maria Letizia
dc.contributor.authorIudici, Michele
dc.contributor.authorMahrhold, Juliane
dc.contributor.authorLocatelli, Francesco
dc.contributor.authorCassone, Giulia
dc.contributor.authorSchiavon, Franco
dc.contributor.authorSeeliger, Benjamin
dc.contributor.authorNeumann, Thomas
dc.contributor.authorFeder, Claudia
dc.contributor.authorKroegel, Claus
dc.contributor.authorGroh, Matthieu
dc.contributor.authorMarvisi, Chiara
dc.contributor.authorSamson, Maxime
dc.contributor.authorBarba, Thomas
dc.contributor.authorJayne, David
dc.contributor.authorTroilo, Arianna
dc.contributor.authorThiel, Jens
dc.contributor.authorHellmich, Bernhard
dc.contributor.authorMonti, Sara
dc.contributor.authorMontecucco, Carlomaurizio
dc.contributor.authorSalvarani, Carlo
dc.contributor.authorKahn, Jean-Emmanuel
dc.contributor.authorBonnotte, Bernard
dc.contributor.authorDurel, Cécile-Audrey
dc.contributor.authorPuéchal, Xavier
dc.contributor.authorMouthon, Luc
dc.contributor.authorGuillevin, Loïc
dc.contributor.authorEmmi, Giacomo
dc.contributor.authorVaglio, Augusto
dc.contributor.authorPorcher, Raphael
dc.contributor.authorTerrier, Benjamin
dc.date.accessioned2024-12-19T13:13:39Z
dc.date.available2024-12-19T13:13:39Z
dc.date.issued2024-12
dc.description.abstractObjectives: Current guidelines suggest treating poor-prognosis eosinophilic granulomatosis with polyangiitis (EGPA) with a combination of glucocorticoids (GCs) plus cyclophosphamide (CYC). However, there is little data to support the need for the addition of CYC. The objective of this study was to compare GCs plus CYC to GCs alone as induction therapy in poor-prognosis EGPA. Methods: We emulated a target trial using observational data from a European multicenter retrospective data base. We included patients with newly diagnosed EGPA with a 1996 Five Factor Score (FFS) of at least 1, treated with GCs or GCs plus CYC between June 1985 and November 2018. Propensity score analysis was used to adjustfor potential confounders. Primary outcome was relapse at 12months. Secondary outcomes included major relapse at 12months and GC-dependent asthma and/or ear nose and throat (ENT) manifestations at 24months. Results: A total of 209 patients were included: 47 % were male and the mean age at diagnosis was 52 (±16 years); 26 % were treated with GCs alone and 74 % with GCs plus CYC. After adjustment, the risk of relapse (hazard ratio [HR]: 0.24, 95%CI [0.08–0.67], p = 0.007), major relapse (HR: 0.24, 95%CI [0.07–0.85], p = 0.026) and the proportion of GC-dependent asthma and/or ENT manifestations (odds ratio:0.30, 95%CI [0.14–0.66], p = 0.003) were lower in the GCs plus CYC group compared to the GCs alone group. Conclusion: This target trial emulation study shows that the addition of CYC to GCs reduces the risk of vasculitis relapse and the rate of GC-dependent asthma and/or ENT manifestations in patients with poor-prognosis EGPA.eng
dc.identifier.doi10.1016/j.jaut.2024.103338
dc.identifier.issn0896-8411
dc.identifier.urihttp://hdl.handle.net/10400.1/26517
dc.language.isoeng
dc.peerreviewedyes
dc.publisherElsevier
dc.relation.hasversionhttps://www.sciencedirect.com/science/article/abs/pii/S0896841124001720
dc.relation.ispartofJournal of Autoimmunity
dc.rights.uriN/A
dc.subjectANCA-Associated vasculitides
dc.subjectCyclophosphamide
dc.subjectEosinophilic Granulomatosis with polyangiitis
dc.subjectPoor-prognosis factor
dc.titleGlucocorticoids versus glucocorticoids plus cyclophosphamide in eosinophilic granulomatosis with polyangiitis with poor-prognosis factorseng
dc.typejournal article
dspace.entity.typePublication
oaire.citation.startPage103338
oaire.citation.titleJournal of Autoimmunity
oaire.citation.volume149
oaire.versionhttp://purl.org/coar/version/c_970fb48d4fbd8a85

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