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Cystic fibrosis: desensitization in delayed hypersensitivity reactions to elexacaftor/tezacaftor/ivacaftor.
Gómez-Ganda, L; Galván-Blasco, P; Fernández-Polo, A; Cardona, V; García-Palop, B; Parramón-Teixidó, C J; Polverino, E; Álvarez-Fernández, A.
Afiliación
  • Gómez-Ganda L; Pharmacy Department, Vall d'Hebron University Hospital, Barcelona, Spain.
  • Galván-Blasco P; Allergology Department, Vall d'Hebron University Hospital, Barcelona, Spain.
  • Fernández-Polo A; Vall Hebron Institut de Recerca (VHIR), Vall d'Hebron University Hospital, Barcelona, Spain.
  • Cardona V; Pharmacy Department, Vall d'Hebron University Hospital, Barcelona, Spain.
  • García-Palop B; Allergology Department, Vall d'Hebron University Hospital, Barcelona, Spain.
  • Parramón-Teixidó CJ; Vall Hebron Institut de Recerca (VHIR), Vall d'Hebron University Hospital, Barcelona, Spain.
  • Polverino E; Pharmacy Department, Vall d'Hebron University Hospital, Barcelona, Spain.
  • Álvarez-Fernández A; Pharmacy Department, Vall d'Hebron University Hospital, Barcelona, Spain.
Front Pharmacol ; 15: 1392986, 2024.
Article en En | MEDLINE | ID: mdl-38933680
ABSTRACT

Background:

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.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Idioma: En Revista: Front Pharmacol Año: 2024 Tipo del documento: Article País de afiliación: España Pais de publicación: Suiza
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
Buscar en Google

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Idioma: En Revista: Front Pharmacol Año: 2024 Tipo del documento: Article País de afiliación: España Pais de publicación: Suiza