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Efficacy and safety profile of biotechnological agents and Janus kinase inhibitors in VEXAS syndrome

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dc.creatorCantarini, Lucaspa
dc.creatorFabiani, Claudiaspa
dc.creatorFrediani, Brunospa
dc.creatorBalistreri, Albertospa
dc.creatorDe La Torre Cifuentes, Ligia Alejandraspa
dc.creatorWiesik-Szewczyk, Ewaspa
dc.creatorOzen, Sezaspa
dc.creatorDeniz Batu, Ezgispa
dc.creatorMaher, Aminaspa
dc.creatorRagab, Gaafarspa
dc.creatorJaber, Nourspa
dc.creatorPortincasa, Pierospa
dc.creatorLa Torre, Francescospa
dc.creatorConticini, Edoardospa
dc.creatorRovera, Guidospa
dc.creatorVitetta, Rosettaspa
dc.creatorBixio, Riccardospa
dc.creatorViapiana, Ombrettaspa
dc.creatorGurnari, Carmelospa
dc.creatorTriggianese, Paolaspa
dc.creatorSoto-Peleteiro, Adrianaspa
dc.creatorRuiz-Irastorza, Guillermospa
dc.creatorMontecucco, Carlomauriziospa
dc.creatorMonti, Saraspa
dc.creatorIannone, Florenzospa
dc.creatorLopalco, Giuseppespa
dc.creatorAlves Cordeiro, Rafaelspa
dc.creatorMayrink Giardini, Henrique A.spa
dc.creatorMormile, Ilariaspa
dc.creatorDe Paulis, Amatospa
dc.creatorD’Agostino, Maria Antoniettaspa
dc.creatorCauli, Albertospa
dc.creatorPiga, Matteospa
dc.creatorKucuk, Hamitspa
dc.creatorTufan, Abdurrahmanspa
dc.creatorSota, Jurgenspa
dc.creatorBaggio, Chiaraspa
dc.creatorBindoli, Saraspa
dc.creatorSfriso, Paolospa
dc.creatorAraújo, Olgaspa
dc.creatorGómez-Caverzaschi, Verónicaspa
dc.creatorHernández-Rodríguez, Joséspa
dc.creatorCrisafulli, Francescaspa
dc.creatorFranceschini, Francospa
dc.creatorFrassi, Micolspa
dc.creatorTomelleri, Alessandrospa
dc.creatorCampochiaro, Corradospa
dc.creatorDagna, Lorenzospa
dc.creatorBeecher, Markspa
dc.creatorCallisto, Aliciaspa
dc.creatorHissaria, Pravinspa
dc.creatorKawakami-Campos, Perla Ayumispa
dc.creatorTorres-Ruiz, Jiramspa
dc.creatorGuaracha-Basañez, Guillermo Arturospa
dc.creatorMartín-Nares, Eduardospa
dc.creatorHinojosa-Azaola, Andreaspa
dc.creatorLeone, Flaviaspa
dc.creatorCaggiano, Valeriaspa
dc.creatorVitale, Antoniospa
dc.date.accessioned2025-07-21T16:40:50Z
dc.date.available2025-07-21T16:40:50Z
dc.date.created2025-01-01spa
dc.date.issued2025-01-01spa
dc.description.abstractBackground: VEXAS syndrome, a recently identified systemic autoinflammatory disorder, poses new diagnostic and management challenges. Based on experience with other autoinflammatory diseases, anti-interleukin (IL)-1, anti-IL-6, anti-tumor necrosis factor (TNF) biotechnological agents, and Janus kinase inhibitors (JAKis) have been widely employed in VEXAS patients. The aim of this study is to evaluate the global effectiveness and safety of biotechnological agents and JAKis using data from the real-world context. Methods: Clinical, laboratory, and therapeutic data from VEXAS patients were obtained from the international AIDA Network VEXAS registry. Results: In total, 69 VEXAS patients were enrolled in the study. Among them, 12 patients (13 treatment courses) received IL-1 inhibitors, 12 patients (13 treatment courses) were administered anti-IL-6 agents, 8 patients (9 treatment courses) were treated with anti-TNF agents, and 16 patients (17 treatment courses) were treated with JAKis. A complete response was observed in 3 patients (23%) treated with anti-IL-1 agents, 2 patients (15%) receiving IL-6 inhibitors, 1 patient (11%) receiving TNF inhibitors, and 4 patients (23.5%) treated with JAKis. The mean prednisone (or equivalent) dosage significantly decreased during anti-IL-1 treatment (p = 0.01), while glucocorticoids changed during anti-IL-6, anti-TNF, and JAKi treatment in a non-significant fashion. A total of 21 patients experienced adverse events, 3 of which led to death (gut perforation, Legionnaires’ disease, and infectious pneumonia) while on JAKis; treatment withdrawal was required for 8 out of 21 patients. Conclusion: IL-1 and IL-6 inhibitors, along with JAKis, represent promising therapeutic options for VEXAS patients, albeit careful monitoring is mandatory to control disease activity and ensure safety.eng
dc.format.mimetypeapplication/pdfspa
dc.identifier.doihttps://doi.org/10.3389/fphar.2025.1462254spa
dc.identifier.issn1663-9812spa
dc.identifier.urihttps://repository.urosario.edu.co/handle/10336/46057
dc.language.isoengspa
dc.publisherFrontiersspa
dc.relation.ispartofFrontiers Pharmacology 18 February 2025 Sec. Inflammation Pharmacology Volume 16 - 2025spa
dc.relation.urihttps://www.frontiersin.org/journals/pharmacology/articles/10.3389/fphar.2025.1462254/fullspa
dc.rightsAttribution-NonCommercial-NoDerivatives 4.0 Internationalspa
dc.rights.accesRightsinfo:eu-repo/semantics/openAccessspa
dc.rights.accesoAbierto (Texto Completo)spa
dc.rights.urihttp://creativecommons.org/licenses/by-nc-sa/4.0/spa
dc.sourceFrontiers Pharmacologyspa
dc.source.instnameinstname:Universidad del Rosariospa
dc.source.reponamereponame:Repositorio Institucional EdocURspa
dc.subject.keywordFarmacologíaeng
dc.subject.keywordAnti-TNFeng
dc.subject.keywordAnakinraeng
dc.subject.keywordCanakinumabeng
dc.subject.keywordJAK inhibitorseng
dc.subject.keywordTocilizumabeng
dc.subject.keywordTreatmenteng
dc.titleEfficacy and safety profile of biotechnological agents and Janus kinase inhibitors in VEXAS syndromespa
dc.typearticlespa
dc.type.hasVersioninfo:eu-repo/semantics/publishedVersionspa
dc.type.spaArtículo de Investigaciónspa
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