Abstract English: Introduction The availability of combination therapy with cystic fibrosis transmembrane conductance regulator (CFTR) modulators, elexacaftor–tezacaftor–ivacaftor (ETI), has significantly improved pulmonary outcomes and quality of life in patients with cystic fibrosis. This study aimed to evaluate structural bronchopulmonary changes using computed tomography (CT) after 1 year of ETI treatment, assessed by the Bhalla score, and to correlate these findings with clinical response parameters. Methods This retrospective observational study included 11 patients under 18 years of age who underwent chest CT before and 1 year after ETI initiation. CT images were assessed using the Bhalla score. Scores range from 0 to 25, with lower values indicating more severe structural lung involvement. CT scores were correlated with changes in sweat test values and percent predicted forced expiratory volume in one second (ppFEV1). Statistical analysis included the paired Student’s t-test, Wilcoxon signed-rank test, and Pearson’s correlation coefficient. Results The mean Bhalla score improved from 14.55 to 18.27 (P < 0.001), with the greatest improvements in mucus plugging (P = 0.002) and peribronchial thickening (P = 0.008). Bronchiectasis improved in some patients but did not reach statistical significance. Structural changes correlated with clinical response: ppFEV1 increased by 13.38% (P = 0.002), while sweat chloride levels decreased by 43.1 mmol/L (P < 0.001). Conclusions ETI therapy produced significant morphological and functional improvements. CT-detected structural changes correlated with clinical outcomes, and the Bhalla score proved useful for monitoring pulmonary disease.
Stănică et al. (Wed,) studied this question.