Elexacaftor-Tezacaftor-Ivacaftor improve Gastro-Oesophageal reflux and Sinonasal symptoms in advanced cystic fibrosis

Upper gastrointestinal and upper airway disease are common in cystic fibrosis (CF) and may contribute to lower airway infection and inflammation. In a longitudinal cohort study of 32 patients (23 men; median age 32.5 years) with advanced CF lung disease (median FEV 1 24.8% predicted) starting elexacaftortezacaftor-ivacaftor, the reflux symptom index score fell from a pre-treatment median (IQR) of 15 (11-23) to 5 (2.8-7.3) (p < 0.001), the Hull airway reflux score fell from a median of 26.5 (16.3-39) to 7.5 (4-12) (p < 0.001), and the sinonasal outcome score from a median of 36.5 (22-24) to 20 (10-32) (p < 0.001) at 6 months on treatment. Mean FEV 1 % predicted rose by 9.2 points, the median respiratory domain score of the CF Questionnaire-Revised rose by 27.8 points and mean body mass index rose by 2.6 kg/m 2 . In addition to improving lung function and weight, CFTR modulators improve upper airway and gastrooesophageal reflux symptoms in advanced CF.(c) 2022 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

Automated summary

Upper gastrointestinal and upper airway diseases are common in cystic fibrosis (CF) and may contribute to lower airway infection and inflammation. A longitudinal cohort study showed that treatment with CFTR modulators can improve respiratory reflux symptoms and sinonasal symptoms, as well as improve lung function and weight in patients with advanced CF.