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

• Published in: Journal of investigational allergology & clinical immunology Vol. 34; no. 3; p. 211
• Main Authors: Mir-Ihara, P, De Las Vecillas, L, Heredia, R, Fiandor, A, González-Muñoz, M, Zamarrón, E, Prados, C, Cabañas, R
• Format: Journal Article
• Language: English
• Published: Spain Esmon Publicidad 17.06.2024
• Subjects: Adult; Aminophenols - adverse effects; Aminophenols - therapeutic use; Benzodioxoles - adverse effects; Benzodioxoles - therapeutic use; Comorbidity; Cystic fibrosis; Cystic Fibrosis - drug therapy; Cystic Fibrosis - immunology; Desensitization, Immunologic - methods; Drug Combinations; Drug Hypersensitivity - diagnosis; Drug Hypersensitivity - immunology; Drug Hypersensitivity - therapy; Eosinophilia; Female; Humans; Hypersensitivity (delayed); Hypersensitivity, Delayed - diagnosis; Hypersensitivity, Delayed - immunology; Indoles - adverse effects; Indoles - therapeutic use; Lung diseases; Lymphocyte Activation - drug effects; Lymphocyte transformation; Lymphocytes; Male; Pyrazoles - adverse effects; Pyrazoles - therapeutic use; Pyridines - adverse effects; Pyridines - therapeutic use; Pyrroles - adverse effects; Pyrrolidines; Quinolines - adverse effects; Quinolines - therapeutic use; Quinolones - adverse effects; Quinolones - therapeutic use; Respiratory function; Cystic fibrosis transmembrane conductance modulators; Cystic fibrosis; Delayed hypersensitivity; Desensitization; Lymphocyte transformation test
• ISSN: 1018-9068; 1698-0808
• DOI: 10.18176/jiaci.0961

Mir-Ihara et al present a case report of successful desensitization to the CFTR modulators ivacaftor and elexacaftor-tezacaftor-ivacaftor (ETI) in a patient with cystic fibrosis (CF) who experienced a delayed hypersensitivity reaction. The patient, a 40-year-old man with severe lung disease, started treatment with CFTR modulators but developed a maculopapular rash and eosinophilia. The treatment was discontinued, and the patient was referred to the allergy unit. Patch tests and a lymphocyte transformation test (LTT) confirmed sensitization to ivacaftor and ETI. Based on the positive LTT result and the patient's comorbidities, a desensitization protocol was initiated. The protocol involved gradually increasing the dose of ivacaftor and ETI over time. The patient tolerated the treatment well and experienced significant improvement in lung function and weight gain. The authors propose this desensitization protocol as a safe and effective method for restarting and continuing treatment with CFTR modulators in patients with delayed hypersensitivity reactions.