Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2026
Effect of discontinuation and reduction of respiratory cotherapies on the effectiveness of CFTR modulators.
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Reynaud Q, Payet C, Fernandez V, Eid E, Bessou A, Belhassen M, Durieu I, Viprey M, investigator group of PHRC
Аннотация
BACKGROUND: We evaluated whether the initiation of CFTR modulators led to a concomitant reduction in or discontinuation of respiratory cotherapies in French pwCF treated with IVA or LUM/IVA and determined whether the reduction in or discontinuation of respiratory cotherapies led to decreased effectiveness of CFTR modulators on clinical status.
METHODS: We conducted a national retrospective cohort study using data from the French CF Registry (FCFR) linked to the French National Health Data System (SNDS), including all French pwCF aged 6 years and older who initiated IVA or LUM/IVA between 2012 and 2020 and who were treated for at least six months. Exposure to respiratory cotherapies was defined as at least one dispensing event of azithromycin, RhDNase, or inhaled antibiotics (aztreonam, colistimethate, or tobramycin).
RESULTS: In total, 1378 pwCF were included in the study, with >90% of the population treated with LUM/IVA. Dornase alpha was discontinued in 25% of adults and 17% of children. Azithromycin was discontinued in 19% of adults and children. For inhaled antibiotics, discontinuation of the three treatments was observed in almost 40% of adults. No effect of the discontinuation of respiratory cotherapy was detected on the FEV1%, number of intravenous antibiotic courses or number of total antibiotic courses (0.13, 95% CI (-1.44; 1.70)); the values were -0.03 (-0.18; 0.13) and 0.04 (-0.25; 0.32), respectively.
CONCLUSION: This real-world study revealed a high rate of respiratory cotherapy discontinuation or reduction during the year following LUM/IVA or IVA initiation, without measurable clinical impact.
METHODS: We conducted a national retrospective cohort study using data from the French CF Registry (FCFR) linked to the French National Health Data System (SNDS), including all French pwCF aged 6 years and older who initiated IVA or LUM/IVA between 2012 and 2020 and who were treated for at least six months. Exposure to respiratory cotherapies was defined as at least one dispensing event of azithromycin, RhDNase, or inhaled antibiotics (aztreonam, colistimethate, or tobramycin).
RESULTS: In total, 1378 pwCF were included in the study, with >90% of the population treated with LUM/IVA. Dornase alpha was discontinued in 25% of adults and 17% of children. Azithromycin was discontinued in 19% of adults and children. For inhaled antibiotics, discontinuation of the three treatments was observed in almost 40% of adults. No effect of the discontinuation of respiratory cotherapy was detected on the FEV1%, number of intravenous antibiotic courses or number of total antibiotic courses (0.13, 95% CI (-1.44; 1.70)); the values were -0.03 (-0.18; 0.13) and 0.04 (-0.25; 0.32), respectively.
CONCLUSION: This real-world study revealed a high rate of respiratory cotherapy discontinuation or reduction during the year following LUM/IVA or IVA initiation, without measurable clinical impact.