Background: Chronic rhinosinusitis (CRS) is prevalent in people with cystic fibrosis (PwCF). Elexacaftor/tezacaftor/ivacaftor (ETI) improves pulmonary disease and CRS in PwCF. Correlations between improvements in quality of life (QOL) and objective measures of CRS severity are understudied in PwCF.
Methods: Adult PwCF/CRS with genotype F508del/F508del or F508del/minimal function participated in a prospective, observational study. Sinus computed tomography (CT) images and 22-question Sinonasal Outcome Test (SNOT-22) questionnaires were obtained at baseline and after 6 months of ETI. CT images were analyzed in a blinded fashion via the Lund Mackay (LM) system and via a deep learning algorithm that quantified the percent of sinus opacification (%SO). Correlations between changes in SNOT-22 total/domain scores and changes in CT scores were evaluated.
Results: 25 PwCF/CRS completed the study. Mean LM, %SO and SNOT-22 total scores were elevated at baseline and improved at follow-up (all p<0.01). Improvement in %SO was not correlated with improvement in SNOT-22 total or domain scores. Improvement in LM score was correlated with improvements in the SNOT-22 ear/facial pain domain (r=-0.43, p=0.03) and trended toward correlation with improvement in SNOT-22 total scores (r=-0.39, p=0.054) and sleep dysfunction domain scores (r=-0.39, p=0.055).
Conclusions: Both sinonasal QOL and sinus CT scores improve after the initiation of highly effective modulator therapy. However, only variable correlations exist between improvement in radiologic and patient-reported CRS severity. Optimization of both sinonasal symptoms and objective sinus inflammation is important for PwCF given lower airway effects that stem from upper airway disease.