Primary Humoral Immunodeficiencies and Bronchiectasis in Adults

Primary humoral immunodeficiencies are a heterogeneous group of disorders defined by quantitative and/or functional defects in one or more immunoglobulin classes, often with associated cellular immune abnormalities. Their link with bronchiectasis, whose prevalence varies across specific defects, is...

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Autores: Suárez Cuartín, Guillermo Rafael, Lores, Carmen, Gómez Olivas, José Daniel, Oscullo, Grace, Martínez García, Miguel Ángel
Formato: artículo
Estado:Versión publicada
Fecha de publicación:2025
País:España
Recursos:Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya)
Repositorio:Recercat. Dipósit de la Recerca de Catalunya
OAI Identifier:oai:recercat.cat:2445/227065
Acesso em linha:https://hdl.handle.net/2445/227065
Access Level:acceso embargado
Palavra-chave:Malalties bronquials
Síndrome del destret respiratori de l'adult
Bronchial diseases
Adult respiratory distress syndrome
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spelling Primary Humoral Immunodeficiencies and Bronchiectasis in AdultsSuárez Cuartín, Guillermo RafaelLores, CarmenGómez Olivas, José DanielOscullo, GraceMartínez García, Miguel ÁngelMalalties bronquialsSíndrome del destret respiratori de l'adultBronchial diseasesAdult respiratory distress syndromePrimary humoral immunodeficiencies are a heterogeneous group of disorders defined by quantitative and/or functional defects in one or more immunoglobulin classes, often with associated cellular immune abnormalities. Their link with bronchiectasis, whose prevalence varies across specific defects, is largely driven by recurrent respiratory infections. Selective Immunoglobulin-(Ig)A deficiency and IgG2 subclass deficiency are the most frequent forms, but common variable immunodeficiency (CVID) is the condition most often associated with bronchiectasis and is usually diagnosed earlier because of its characteristic phenotype. In contrast, the contribution of isolated IgA deficiency or selective IgG subclass deficiencies to bronchiectasis remains controversial. Other reported associations include X-linked agammaglobulinemia, selective IgM or IgG deficiency, and rarer entities such as selective IgE deficiency, unclassified hypogammaglobulinemia, specific antibody deficiency, specific polysaccharide antibody deficiency, and heavy- or light-chain deficiencies. Current bronchiectasis guidelines recommend measurement of serum immunoglobulins and IgG subclasses in patients with compatible features, recurrent infections, or no clear etiology before labeling disease as idiopathic. Identifying immunoglobulin defects is clinically important because they represent treatable traits. The potential role of emerging therapies such as the DPP1 inhibitor brensocatib in immunodeficiency-related bronchiectasis remains uncertain, and ongoing registries will be key to clarifying these relationships.MDPI AG2026202620252026info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersion16 p.application/pdfhttps://hdl.handle.net/2445/227065Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL))reponame:Recercat. Dipósit de la Recerca de Catalunyainstname:Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya)InglésReproducció del document publicat a: https://doi.org/10.3390/jcm15010179Journal of Clinical Medicine, 2025, vol. 15, issue. 1, p. 179https://doi.org/10.3390/jcm15010179https://creativecommons.org/licenses/by/4.0/info:eu-repo/semantics/embargoedAccessoai:recercat.cat:2445/2270652026-05-29T05:05:01Z
dc.title.none.fl_str_mv Primary Humoral Immunodeficiencies and Bronchiectasis in Adults
title Primary Humoral Immunodeficiencies and Bronchiectasis in Adults
spellingShingle Primary Humoral Immunodeficiencies and Bronchiectasis in Adults
Suárez Cuartín, Guillermo Rafael
Malalties bronquials
Síndrome del destret respiratori de l'adult
Bronchial diseases
Adult respiratory distress syndrome
title_short Primary Humoral Immunodeficiencies and Bronchiectasis in Adults
title_full Primary Humoral Immunodeficiencies and Bronchiectasis in Adults
title_fullStr Primary Humoral Immunodeficiencies and Bronchiectasis in Adults
title_full_unstemmed Primary Humoral Immunodeficiencies and Bronchiectasis in Adults
title_sort Primary Humoral Immunodeficiencies and Bronchiectasis in Adults
dc.creator.none.fl_str_mv Suárez Cuartín, Guillermo Rafael
Lores, Carmen
Gómez Olivas, José Daniel
Oscullo, Grace
Martínez García, Miguel Ángel
author Suárez Cuartín, Guillermo Rafael
author_facet Suárez Cuartín, Guillermo Rafael
Lores, Carmen
Gómez Olivas, José Daniel
Oscullo, Grace
Martínez García, Miguel Ángel
author_role author
author2 Lores, Carmen
Gómez Olivas, José Daniel
Oscullo, Grace
Martínez García, Miguel Ángel
author2_role author
author
author
author
dc.subject.none.fl_str_mv Malalties bronquials
Síndrome del destret respiratori de l'adult
Bronchial diseases
Adult respiratory distress syndrome
topic Malalties bronquials
Síndrome del destret respiratori de l'adult
Bronchial diseases
Adult respiratory distress syndrome
description Primary humoral immunodeficiencies are a heterogeneous group of disorders defined by quantitative and/or functional defects in one or more immunoglobulin classes, often with associated cellular immune abnormalities. Their link with bronchiectasis, whose prevalence varies across specific defects, is largely driven by recurrent respiratory infections. Selective Immunoglobulin-(Ig)A deficiency and IgG2 subclass deficiency are the most frequent forms, but common variable immunodeficiency (CVID) is the condition most often associated with bronchiectasis and is usually diagnosed earlier because of its characteristic phenotype. In contrast, the contribution of isolated IgA deficiency or selective IgG subclass deficiencies to bronchiectasis remains controversial. Other reported associations include X-linked agammaglobulinemia, selective IgM or IgG deficiency, and rarer entities such as selective IgE deficiency, unclassified hypogammaglobulinemia, specific antibody deficiency, specific polysaccharide antibody deficiency, and heavy- or light-chain deficiencies. Current bronchiectasis guidelines recommend measurement of serum immunoglobulins and IgG subclasses in patients with compatible features, recurrent infections, or no clear etiology before labeling disease as idiopathic. Identifying immunoglobulin defects is clinically important because they represent treatable traits. The potential role of emerging therapies such as the DPP1 inhibitor brensocatib in immunodeficiency-related bronchiectasis remains uncertain, and ongoing registries will be key to clarifying these relationships.
publishDate 2025
dc.date.none.fl_str_mv 2025
2026
2026
2026
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv https://hdl.handle.net/2445/227065
url https://hdl.handle.net/2445/227065
dc.language.none.fl_str_mv Inglés
language_invalid_str_mv Inglés
dc.relation.none.fl_str_mv Reproducció del document publicat a: https://doi.org/10.3390/jcm15010179
Journal of Clinical Medicine, 2025, vol. 15, issue. 1, p. 179
https://doi.org/10.3390/jcm15010179
dc.rights.none.fl_str_mv https://creativecommons.org/licenses/by/4.0/
info:eu-repo/semantics/embargoedAccess
rights_invalid_str_mv https://creativecommons.org/licenses/by/4.0/
eu_rights_str_mv embargoedAccess
dc.format.none.fl_str_mv 16 p.
application/pdf
dc.publisher.none.fl_str_mv MDPI AG
publisher.none.fl_str_mv MDPI AG
dc.source.none.fl_str_mv Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL))
reponame:Recercat. Dipósit de la Recerca de Catalunya
instname:Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya)
instname_str Varias* (Consorci de Biblioteques Universitáries de Catalunya, Centre de Serveis Científics i Acadèmics de Catalunya)
reponame_str Recercat. Dipósit de la Recerca de Catalunya
collection Recercat. Dipósit de la Recerca de Catalunya
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repository.mail.fl_str_mv
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