Chronic rhinosinusitis (CRS) is the most common upper airway disorder in patients with cystic fibrosis (CF), characterized by nasal obstruction, infections and impaired quality of life (QoL). In a unified airway model, paranasal sinuses can serve as a reservoir for bacteria that can lead to pulmonary exacerbations. Elexacaftor/tezacaftor/ivacaftor (ETI) is a CFTR modulator therapy available for CF patients aged 6 or older, who have at least one responsive mutation. Our purpose is to evaluate the impact of ETI on CRS in pediatric CF patients. This observational on-going cohort study includes patients aged 12-18 years eligible for ETI therapy. Patients undergo an evaluation before starting ETI, 3 and 6 months from the beginning. Each visit includes nasal endoscopy, assessment of rhinologic symptoms, QoL and respiratory function test (RFT). Endoscopic score is determined using the modified Lund-Kennedy scoring system, symptoms are assessed with the Sinus and Nasal Quality of Life Survey for pediatric patients (SNOT-5). Preliminary results of the first 10 patients analyzed are shown in Table1. Endoscopic and SNOT-5 scores decreased, whereas QoL score and respiratory function increased, all with a statistically significant improvement. From the first months, ETI reduces endoscopic score and symptoms and improves RFT  and QoL in CF children with CRS.

Table 1: results at baseline, at 3 and 6 months of the first 10 patients, RFT not showed.