Protocol for Successful Desensitization to Ivacaftor and Elexacaftor/Tezacaftor/Ivacaftor in a Delayed Hypersensitivity Reaction Confirmed by the Lymphocyte Transformation Test

Mir-Ihara, P; De Las Vecillas, L; Heredia, R; Fiandor, A; González-Muñoz, M; Zamarrón, E; Prados, C; Cabañas, R

doi:10.18176/jiaci.0961

Cited by 1 publication

(1 citation statement)

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“…One patient had positive patch tests to ELX/TEZ/IVA and IVA, confirming a type IV hypersensitivity, which aligns with findings reported by Balijepally et al, 2022 . Recently, Mir-Ihara et al, 2023 have reported a case where a man presented a maculopapular rash and eosinophilia seven days into treatment with ELX/TEZ/IVA and underwent epicutaneous patch tests with ELX/TEZ/IVA and IVA in 30% petrolatum, showing negative readings at 48, 72, and 96 h.…”

Section: Discussionmentioning

confidence: 99%

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

Gómez-Ganda,

Galván-Blasco,

Fernández-Polo

et al. 2024

Front. Pharmacol.

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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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Section: Discussionmentioning

confidence: 99%