Revue medicale de Liege · 2026
[Evolution of thoracic computed tomography after therapy with elexacaftor, tezacaftor, and ivacaftor in patients with cystic fibrosis].
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Thimmesch Matthieu, Cheping Simo Christian, Boulay Matthieu, Pirson Jessica, Palem Alain
Аннотация
Cystic fibrosis, the most common genetic disorder in Caucasian populations, is caused by mutations in the CFTR gene (Cystic Fibrosis Transmembrane Conductance Regulator), which disrupts chloride ion transport and leads to the production of viscous mucus, primarily affecting the lungs, digestive system, and liver. Traditional symptomatic treatment has been improved with the advent of CFTR modulators, notably the combination therapy of elexacaftor, tezacaftor, and ivacaftor (ETI), for patients carrying at least one F508del mutation. This retrospective study includes 23 patients treated with ETI, analyzing thoracic CT scan results before and after treatment using the Bhalla score. The results demonstrate a significant improvement in the Bhalla score (17.2 (± 3.8) before ETI vs 18.3 (± 3.4) after ETI, p < 0.001). The score improvement was observed in 78 % of patients, while 13 % remained stable, and 9 % showed deterioration. These results are consistent with other studies, although the effect on the improvement of bronchiectasis remains uncertain. Despite promising results, the study remains limited by its monocentric design and small sample size. Nevertheless, it confirms the beneficial effect of ETI on radiological pulmonary damage in CF patients.