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Immunologic biomarkers of noninfectious complications and overall survival in common variable immunodeficiency

2026-02Artigo científico Acesso aberto
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dc.contributor.authorTorres-Valle, Alba
dc.contributor.authorNeirinck, Jana
dc.contributor.authorSilva, Susana L.
dc.contributor.authorde Arriba, Sonia
dc.contributor.authorSerrano, Cristina
dc.contributor.authorCordón, Lourdes
dc.contributor.authorArenas-Caro, Pedro Pablo
dc.contributor.authorSubirá, Dolores
dc.contributor.authorMercado, Marta Ruiz
dc.contributor.authorGonzález Granado, Luis I.
dc.contributor.authorMartins, Catarina
dc.contributor.authorKalina, Tomas
dc.contributor.authorBalastegui-Martín, Héctor
dc.contributor.authorCabanero-Navalon, Marta Dafne
dc.contributor.authorMarcos, Miguel
dc.contributor.authorInes, Sandra
dc.contributor.authorContreras, Teresa
dc.contributor.authorPrieto, Carlos
dc.contributor.authorGuerrero, Lucía
dc.contributor.authorGarcía Cañadas, M. Carmen
dc.contributor.authorCordeiro, Ana Isabel
dc.contributor.authorAlbarran, Beatriz
dc.contributor.authorGómez, Nicolás González
dc.contributor.authorHurtado, Guillermina
dc.contributor.authorBarez, Abelardo
dc.contributor.authorMadruga, Ignacio
dc.contributor.authorGarcía-Sancho, Alejandro Martin
dc.contributor.authorBastida, José María
dc.contributor.authorDavila, Ignacio
dc.contributor.authorAragon, Larraitz
dc.contributor.authorRequejo Olaizola, Pedro Mikel
dc.contributor.authorHofmans, Mattias
dc.contributor.authorDe Vriendt, Ciel
dc.contributor.authorBlanco, Elena
dc.contributor.authorJara-Acevedo, María
dc.contributor.authorKerre, Tessa
dc.contributor.authorSempere, Amparo
dc.contributor.authorHaerynck, Filomeen
dc.contributor.authorPrada, Álvaro
dc.contributor.authorSousa, Ana E.
dc.contributor.authorBonroy, Carolien
dc.contributor.authorvan Dongen, Jacques J.M.
dc.contributor.authorPérez-Andrés, Martín
dc.contributor.authorOrfao, Alberto
dc.contributor.institutionNOVA Medical School|Faculdade de Ciências Médicas (NMS|FCM)
dc.contributor.institutionComprehensive Health Research Centre (CHRC) - pólo NMS
dc.contributor.pblMOSBY-ELSEVIER
dc.date.accessioned2026-01-14T15:51:48Z
dc.date.available2026-01-14T15:51:48Z
dc.date.issued2026-02
dc.descriptionPublisher Copyright: © 2025 The Authors
dc.description.abstractBackground: Common variable immunodeficiency (CVID) includes a heterogeneous group of disorders of predominantly antibody deficiencies featuring infectious and noninfectious complications that might lead to severe organ damage and shortened survival. Appropriate clinical management of CVID has been hampered by the lack of robust biomarkers to predict the development of clinical complications and patient outcome. Objective: We investigated the association of individual serologic, cellular, and molecular biomarkers with disease behavior and outcome in CVID. Methods: A multicenter cohort of 209 CVID patients was studied using age-matched reference values from 334 healthy donors to better define TCD4+-naive cell defects (late-onset combined immunodeficiency [LOCID]) and classify CVID-associated B-cell/plasma cell (PC) and natural killer (NK) cell defects. Results: Globally, susceptibility to respiratory infections was strongly associated with low serum immunoglobulin (sIg), particularly sIgA, whereas noninfectious complications and disease severity mostly depended on TCD4+-naive cell, NK cell, and B-cell/PC defects. LOCID was independently associated with splenomegaly, lymphadenopathy, interstitial lung disease, cytopenia, and lymphoma. Milder B-cell/PC defects (MBC+/PC+/Ab−) protected from noninfectious complications, whereas a marked defect of classical CD27+ memory B cells (27MBC−) (with decreased NK cell and sIgM) was associated with enteropathy and (with LOCID and sIgA) liver disease. Together, lower sIgG, LOCID, and particularly 27MBC−, were strongly associated with shorter survival and early death in CVID. Conversely, CVID-associated pathogenic/risk alleles did not emerge as independent factors associated with disease behavior and outcome. Conclusion: Our results provide a new set of biomarkers closely associated with infectious and noninfectious complications of CVID, which together predict survival and might contribute to guide patient monitoring and clinical management.en
dc.description.versionpublishersversion
dc.description.versionpublished
dc.format.extent2086685
dc.identifier.doi10.1016/j.jaci.2025.10.020
dc.identifier.issn0091-6749
dc.identifier.otherPURE: 147312590
dc.identifier.otherPURE UUID: cebae913-d6e3-4ba9-b783-fff3b8831dbb
dc.identifier.otherScopus: 105022491291
dc.identifier.otherPubMed: 41176068
dc.identifier.otherORCID: /0000-0003-0353-0421/work/207209365
dc.identifier.urihttp://hdl.handle.net/10362/198995
dc.identifier.urlhttps://www.scopus.com/pages/publications/105022491291
dc.language.isoeng
dc.peerreviewedyes
dc.subjectCommon variable immunodeficiency
dc.subjectCVID
dc.subjectdisease complications
dc.subjectimmunoglobulins
dc.subjectlate-onset combined immunodeficiency
dc.subjectmemory B cells
dc.subjectNK cells
dc.subjectplasma cells
dc.subjectsurvival
dc.subjectTCD4-naïve cells
dc.subjectImmunology and Allergy
dc.subjectImmunology
dc.titleImmunologic biomarkers of noninfectious complications and overall survival in common variable immunodeficiencyen
dc.typejournal article
degois.publication.firstPage454
degois.publication.issue2
degois.publication.lastPage469
degois.publication.titleJournal Of Allergy And Clinical Immunology
degois.publication.volume157
dspace.entity.typePublication
rcaap.rightsopenAccess

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