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. 2025 Sep 11:S1569-1993(25)01579-6.
doi: 10.1016/j.jcf.2025.08.019. Online ahead of print.

Factors associated with sustained Pseudomonas aeruginosa infection following elexacaftor/tezacaftor/ivacaftor treatment: Real-world data from the European cystic fibrosis society patient registry

Mordechai Pollak  1 , Simone Gambazza  2 , Annalisa Orenti  2 , Dario Prais  3 , Eitan Kerem  4 , Meir Mei-Zahav  3 ; ECFSPR Steering Group
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Free article

Factors associated with sustained Pseudomonas aeruginosa infection following elexacaftor/tezacaftor/ivacaftor treatment: Real-world data from the European cystic fibrosis society patient registry

Mordechai Pollak et al. J Cyst Fibros. .
Free article

Abstract

Background: Elexacaftor/tezacaftor/ivacaftor (ETI) therapy reduces airway infection rates with Pseudomonas aeruginosa (PsA). This study assessed factors associated with sustained chronic PsA infection and evaluated clinical outcomes after one year of ETI, stratified by PsA status.

Methods: Using the European Cystic Fibrosis Society Patient Registry (ECFSPR), we identified people with cystic fibrosis (pwCF) who initiated ETI between 2019 and 2022. Multivariable logistic regression was used to identify clinical and demographic predictors of persistent chronic PsA. Changes in clinical outcomes from one year before to one year after ETI were analyzed using semiparametric models.

Results: Among 8188 pwCF with chronic PsA prior to ETI, 4908 (59.9 %) remained chronically infected after one year, while 3280 (40.1 %) transitioned to non-chronic PsA. Older age (OR 1.63, 95 %CI: 1.49-1.78) and lower baseline ppFEV₁ (OR 0.64, 95 %CI: 0.58-0.70) were the strongest predictors of persistent chronic PsA. The improvement in ppFEV₁ was similar between those who remained chronic (12.5 %, 95 %CI: 11.8-13.3) and those who shifted to non-chronic status (13.0 %, 95 %CI: 12.2-13.8; p = 0.074). BMI z-score improvements did not differ significantly between groups (p = 0.086). However, the mean hospital admission days was lower in those who shifted to non-chronic PsA (1.2 vs. 1.9 days; p < 0.001).

Conclusion: Risk factors for sustained PsA infection post-ETI are associated with older age and lower ppFEV1. Nonetheless, improvements in lung function and nutritional status were comparable, regardless of PsA status. Further research is required to better understand how PsA affects CF lung disease in the era of ETI treatment.

Keywords: BMI; CFTR modulators; ETI; Elexacaftor/ tezacaftor/ ivacaftor; FEV1; Pseudomonas Aeruginosa; Registry; Trikafta; kaftrio.

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Conflict of interest statement

Declaration of competing interest DP declares the following financial interests/personal relationships which may be considered as potential competing interests: Grant/Research Support: Circle of care educational grant by Vertex Principal investigator for Vertex studies: VX-445–113, VX-445–116, VX-445–119, VX-121–102, VX-121–103, VX-121–104 Speaker’s Bureau: AstraZeneca, Sanofi, Trupharm, Vertex Consultant: Vertex - Global Principal Investigator VX-121–104 All other authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.

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