Abstract
Introduction: Elexacaftor-tezacaftor-ivacaftor (ETI) significantly improves lung function in CF patients. Previous studies have estimated that a delay in ETI initiation could have a negative impact on long-term survival. ETI use has been approved for children aged ≥6 years fulfilling the genetic criteria independent of their lung function. However, there is still controversy regarding ETI use for adult patients with preserved lung function [defined as a percentage of predicted forced expiratory volume in the first second (ppFEV1)>90%] considering that this subgroup of patients was excluded in the screening period of phase III randomized clinical trials.
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