Resident Physician University of Arkansas for Medical Sciences
Introduction: Cystic fibrosis transmembrane conductance regulator (CFTR) modulators have been shown to improve SNOT-22 scores in patients with Cystic Fibrosis (CF) but data assessing objective measures in this patient population is lacking. While it has been argued by some authors that computed tomography (CT) scans are not reliable in determining the need for surgery in patients with CF, imaging of the paranasal sinuses could be useful in providing an objective measure of response to combination CFTR modulator therapy.
Methods: This is a single institution retrospective review of patients with CF who were treated with CFTR modulator therapy between 2016 and 2021. All patients who had CT scans available both before and after initiation of CFTR modulator therapy or a change in CFTR modulator therapy were included. Lund-Mackay scores were calculated. Pre and post-therapy scores were compared.
Results: 265 patients with CF were evaluated. Ten patients had CT scans of the paranasal sinuses available prior to and after initiation of or change in CFTR modulator therapy. 9 out of 10 patients showed objective improvement in Lund-Mackay scores with an average improvement of 8 points. This improvement was statistically significant (p=0.0045). The subset of patients that were treated with elexacaftor/tezacaftor/ivacaftor showed significantly more improvement in Lund-Mackay scores compared to patients treated with any other CFTR modulator therapy (15 vs 3 points; p=0.0009).
Conclusion: Patients with CF treated with CFTR modulators showed significant improvement in Lund-Mackay scores with patients treated with elexacaftor/tezacaftor/ivacaftor showing the greatest benefit.