Cystic fibrosis: desensitization in delayed hypersensitivity reactions to elexacaftor/tezacaftor/ivacaftor

Cystic fibrosis; Delayed hypersensitivity; Rash

Bibliographic Details
Authors: Garcia-Palop, Beatriz, Álvarez Fernandez, Antonio, Gómez-Ganda, Laura, GALVÁN BLASCO, PAULA, Fernandez-Polo, Aurora, Cardona, Victoria, Parramón-Teixidó, Carlos Javier, Polverino, Eva
Format: article
Status:Published version
Publication Date:2024
Country:España
Institution:Departament de Salut de la Generalitat de Catalunya (DS)
Repository:Scientia. Dipòsit d'Informació Digital del Departament de Salut
OAI Identifier:oai:scientiasalut.gencat.cat:11351/11657
Online Access:https://hdl.handle.net/11351/11657
Access Level:Open access
Keyword:Fibrosi quística - Tractament
Immunosupressió
Pell - Ferides i lesions
DISEASES::Respiratory Tract Diseases::Lung Diseases::Cystic Fibrosis
ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT::Therapeutics::Biological Therapy::Immunomodulation::Immunotherapy::Immunosuppression::Desensitization, Immunologic
DISEASES::Skin and Connective Tissue Diseases::Skin Diseases::Exanthema
ENFERMEDADES::enfermedades respiratorias::enfermedades pulmonares::fibrosis quística
TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS::terapéutica::terapia biológica::inmunomodulación::inmunoterapia::inmunosupresión::desensibilización inmunológica
ENFERMEDADES::enfermedades de la piel y tejido conjuntivo::enfermedades de la piel::exantema
id ES_ee92e8ee1b5bbaebec843d0d3d63fc06
oai_identifier_str oai:scientiasalut.gencat.cat:11351/11657
network_acronym_str ES
network_name_str España
repository_id_str
dc.title.none.fl_str_mv Cystic fibrosis: desensitization in delayed hypersensitivity reactions to elexacaftor/tezacaftor/ivacaftor
title Cystic fibrosis: desensitization in delayed hypersensitivity reactions to elexacaftor/tezacaftor/ivacaftor
spellingShingle Cystic fibrosis: desensitization in delayed hypersensitivity reactions to elexacaftor/tezacaftor/ivacaftor
Garcia-Palop, Beatriz
Fibrosi quística - Tractament
Immunosupressió
Pell - Ferides i lesions
DISEASES::Respiratory Tract Diseases::Lung Diseases::Cystic Fibrosis
ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT::Therapeutics::Biological Therapy::Immunomodulation::Immunotherapy::Immunosuppression::Desensitization, Immunologic
DISEASES::Skin and Connective Tissue Diseases::Skin Diseases::Exanthema
ENFERMEDADES::enfermedades respiratorias::enfermedades pulmonares::fibrosis quística
TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS::terapéutica::terapia biológica::inmunomodulación::inmunoterapia::inmunosupresión::desensibilización inmunológica
ENFERMEDADES::enfermedades de la piel y tejido conjuntivo::enfermedades de la piel::exantema
title_short Cystic fibrosis: desensitization in delayed hypersensitivity reactions to elexacaftor/tezacaftor/ivacaftor
title_full Cystic fibrosis: desensitization in delayed hypersensitivity reactions to elexacaftor/tezacaftor/ivacaftor
title_fullStr Cystic fibrosis: desensitization in delayed hypersensitivity reactions to elexacaftor/tezacaftor/ivacaftor
title_full_unstemmed Cystic fibrosis: desensitization in delayed hypersensitivity reactions to elexacaftor/tezacaftor/ivacaftor
title_sort Cystic fibrosis: desensitization in delayed hypersensitivity reactions to elexacaftor/tezacaftor/ivacaftor
dc.creator.none.fl_str_mv Garcia-Palop, Beatriz
Álvarez Fernandez, Antonio
Gómez-Ganda, Laura
GALVÁN BLASCO, PAULA
Fernandez-Polo, Aurora
Cardona, Victoria
Parramón-Teixidó, Carlos Javier
Polverino, Eva
author Garcia-Palop, Beatriz
author_facet Garcia-Palop, Beatriz
Álvarez Fernandez, Antonio
Gómez-Ganda, Laura
GALVÁN BLASCO, PAULA
Fernandez-Polo, Aurora
Cardona, Victoria
Parramón-Teixidó, Carlos Javier
Polverino, Eva
author_role author
author2 Álvarez Fernandez, Antonio
Gómez-Ganda, Laura
GALVÁN BLASCO, PAULA
Fernandez-Polo, Aurora
Cardona, Victoria
Parramón-Teixidó, Carlos Javier
Polverino, Eva
author2_role author
author
author
author
author
author
author
dc.contributor.none.fl_str_mv Institut Català de la Salut
[Gómez-Ganda L, Fernández-Polo A, García-Palop B, Parramón-Teixidó CJ] Servei de Farmàcia, Vall d’Hebron Hospital Universitari, Barcelona, Spain. [Galván-Blasco P, Cardona V] Servei d’Al·lergologia, Vall d’Hebron Hospital Universitari, Barcelona, Spain. Vall Hebron Institut de Recerca (VHIR), Barcelona, Spain. [Polverino E, Álvarez-Fernández A] Unitat de Fibrosi Quística, Pneumologia, Vall d’Hebron Hospital Universitari, Barcelona, Spain
Vall d'Hebron Barcelona Hospital Campus
dc.subject.none.fl_str_mv Fibrosi quística - Tractament
Immunosupressió
Pell - Ferides i lesions
DISEASES::Respiratory Tract Diseases::Lung Diseases::Cystic Fibrosis
ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT::Therapeutics::Biological Therapy::Immunomodulation::Immunotherapy::Immunosuppression::Desensitization, Immunologic
DISEASES::Skin and Connective Tissue Diseases::Skin Diseases::Exanthema
ENFERMEDADES::enfermedades respiratorias::enfermedades pulmonares::fibrosis quística
TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS::terapéutica::terapia biológica::inmunomodulación::inmunoterapia::inmunosupresión::desensibilización inmunológica
ENFERMEDADES::enfermedades de la piel y tejido conjuntivo::enfermedades de la piel::exantema
topic Fibrosi quística - Tractament
Immunosupressió
Pell - Ferides i lesions
DISEASES::Respiratory Tract Diseases::Lung Diseases::Cystic Fibrosis
ANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT::Therapeutics::Biological Therapy::Immunomodulation::Immunotherapy::Immunosuppression::Desensitization, Immunologic
DISEASES::Skin and Connective Tissue Diseases::Skin Diseases::Exanthema
ENFERMEDADES::enfermedades respiratorias::enfermedades pulmonares::fibrosis quística
TÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS::terapéutica::terapia biológica::inmunomodulación::inmunoterapia::inmunosupresión::desensibilización inmunológica
ENFERMEDADES::enfermedades de la piel y tejido conjuntivo::enfermedades de la piel::exantema
description Cystic fibrosis; Delayed hypersensitivity; Rash
publishDate 2024
dc.date.none.fl_str_mv 2024
2024
2024
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/11351/11657
url https://hdl.handle.net/11351/11657
dc.language.none.fl_str_mv Inglés
language_invalid_str_mv Inglés
dc.relation.none.fl_str_mv Frontiers in Pharmacology;15
https://doi.org/10.3389/fphar.2024.1392986
dc.rights.none.fl_str_mv Attribution 4.0 International
http://creativecommons.org/licenses/by/4.0/
info:eu-repo/semantics/openAccess
rights_invalid_str_mv Attribution 4.0 International
http://creativecommons.org/licenses/by/4.0/
eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.publisher.none.fl_str_mv Frontiers Media
publisher.none.fl_str_mv Frontiers Media
dc.source.none.fl_str_mv Scientia
reponame:Scientia. Dipòsit d'Informació Digital del Departament de Salut
instname:Departament de Salut de la Generalitat de Catalunya (DS)
instname_str Departament de Salut de la Generalitat de Catalunya (DS)
reponame_str Scientia. Dipòsit d'Informació Digital del Departament de Salut
collection Scientia. Dipòsit d'Informació Digital del Departament de Salut
repository.name.fl_str_mv
repository.mail.fl_str_mv
_version_ 1869423733300527104
spelling Cystic fibrosis: desensitization in delayed hypersensitivity reactions to elexacaftor/tezacaftor/ivacaftorGarcia-Palop, BeatrizÁlvarez Fernandez, AntonioGómez-Ganda, LauraGALVÁN BLASCO, PAULAFernandez-Polo, AuroraCardona, VictoriaParramón-Teixidó, Carlos JavierPolverino, EvaFibrosi quística - TractamentImmunosupressióPell - Ferides i lesionsDISEASES::Respiratory Tract Diseases::Lung Diseases::Cystic FibrosisANALYTICAL, DIAGNOSTIC AND THERAPEUTIC TECHNIQUES, AND EQUIPMENT::Therapeutics::Biological Therapy::Immunomodulation::Immunotherapy::Immunosuppression::Desensitization, ImmunologicDISEASES::Skin and Connective Tissue Diseases::Skin Diseases::ExanthemaENFERMEDADES::enfermedades respiratorias::enfermedades pulmonares::fibrosis quísticaTÉCNICAS Y EQUIPOS ANALÍTICOS, DIAGNÓSTICOS Y TERAPÉUTICOS::terapéutica::terapia biológica::inmunomodulación::inmunoterapia::inmunosupresión::desensibilización inmunológicaENFERMEDADES::enfermedades de la piel y tejido conjuntivo::enfermedades de la piel::exantemaCystic fibrosis; Delayed hypersensitivity; RashFibrosis quística; Hipersensibilidad retardada; ErupciónFibrosi quística; Hipersensibilitat retardada; ErupcióBackground: Cystic fibrosis transmembrane conductance regulator modulators are the only available treatment for cystic fibrosis. Although elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) is well-tolerated, rash has been reported as very frequent. In severe rashes, ELX/TEZ/IVA withdrawal is necessary, leading to clinical deterioration. The objective of the study is to increment the experience of ELX/TEZ/IVA desensitization. Methods: Adult patients who developed a delayed hypersensitivity rash to ELX/TEZ/IVA between December 2021 and February 2023 and required withdrawal due to ineffective rescue medication were included. Skins test for ELX/TEZ/IVA and IVA were conducted to establish hypersensitivity mechanism. Balijepally ELX/TEZ/IVA desensitization protocol was selected. In cases where desensitization had to be discontinued due to rash, an extended desensitization was proposed. Clinical and health-related quality of life parameters were collected before ELX/TEZ/IVA and after desensitization. Results: 162 patients (81 women, 31.2 [23.8–42.5] years) started ELX/TEZ/IVA, developing rash 12 of them (7.4%, six women). Six patients (five women) required stopping ELX/TEZ/IVA and were selected for desensitization. Skin tests indicated delayed type-IV hypersensitivity in one patient. Two patients presented adequate tolerance to desensitization; while, four patients developed rash. Three of these patients, successfully concluded extended desensitization (one patient declined participation). No significant clinical deterioration or quality of life worsening was observed during desensitization; in fact, there was an improvement in practically all mesured parameters. All five patients who resumed ELX/TEZ/IVA are currently receiving therapy with good tolerance. Conclusion: Desensitization to ELX/TEZ/IVA could be a successful and safe strategy for reintroducing this essential treatment in cases of a delayed hypersensitivity rash.Frontiers MediaInstitut Català de la Salut[Gómez-Ganda L, Fernández-Polo A, García-Palop B, Parramón-Teixidó CJ] Servei de Farmàcia, Vall d’Hebron Hospital Universitari, Barcelona, Spain. [Galván-Blasco P, Cardona V] Servei d’Al·lergologia, Vall d’Hebron Hospital Universitari, Barcelona, Spain. Vall Hebron Institut de Recerca (VHIR), Barcelona, Spain. [Polverino E, Álvarez-Fernández A] Unitat de Fibrosi Quística, Pneumologia, Vall d’Hebron Hospital Universitari, Barcelona, SpainVall d'Hebron Barcelona Hospital Campus202420242024info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdfhttps://hdl.handle.net/11351/11657Scientiareponame:Scientia. Dipòsit d'Informació Digital del Departament de Salutinstname:Departament de Salut de la Generalitat de Catalunya (DS)InglésFrontiers in Pharmacology;15https://doi.org/10.3389/fphar.2024.1392986Attribution 4.0 Internationalhttp://creativecommons.org/licenses/by/4.0/info:eu-repo/semantics/openAccessoai:scientiasalut.gencat.cat:11351/116572026-06-12T09:38:37Z
score 15,811543

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