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

J Cyst Fibros. 2022 Sep;21(5):807-810. doi: 10.1016/j.jcf.2022.06.003. Epub 2022 Jun 16.

Abstract

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 FEV1 24.8% predicted) starting elexacaftor-tezacaftor-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 FEV1% 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/m2. In addition to improving lung function and weight, CFTR modulators improve upper airway and gastro-oesophageal reflux symptoms in advanced CF.

Keywords: CFTR modulators; Cystic fibrosis; Gastro-oesophageal reflux; Sinonasal symptoms.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aminophenols / therapeutic use
  • Benzodioxoles
  • Chloride Channel Agonists
  • Cystic Fibrosis Transmembrane Conductance Regulator / genetics
  • Cystic Fibrosis* / complications
  • Cystic Fibrosis* / diagnosis
  • Cystic Fibrosis* / drug therapy
  • Gastroesophageal Reflux* / complications
  • Gastroesophageal Reflux* / diagnosis
  • Gastroesophageal Reflux* / drug therapy
  • Humans
  • Indoles
  • Longitudinal Studies
  • Male
  • Mutation
  • Pyrazoles
  • Pyridines
  • Pyrrolidines
  • Quinolones

Substances

  • Aminophenols
  • Benzodioxoles
  • Chloride Channel Agonists
  • Indoles
  • Pyrazoles
  • Pyridines
  • Pyrrolidines
  • Quinolones
  • tezacaftor
  • Cystic Fibrosis Transmembrane Conductance Regulator
  • ivacaftor
  • elexacaftor