Abstract
BackgroundExtra-oesophageal reflux (EOR) may lead to microaspiration in patients with cystic fibrosis (CF), a probable cause of deteriorating lung function. Successful clinical trials of ivacaftor highlight opportunities to understand EOR in a real world study. MethodsData from 12 patients with CF and the G551D mutation prescribed ivacaftor (150mg bd) was collected at baseline, 6, 26 and 52weeks. The changes in symptoms of EOR were assessed by questionnaire (reflux symptom index (RSI) and Hull airway reflux questionnaire (HARQ)). ResultsSix patients presented EOR at baseline (RSI >13; median 13; range 2–29) and 5 presented airway reflux (HARQ >13; median 12; range 3 to 33). Treatment with ivacaftor was associated with a significant reduction of EOR symptoms (P<0∙04 versus baseline) denoted by the reflux symptom index and Hull airway reflux questionnaire. ConclusionIvacaftor treatment was beneficial for patients with symptoms of EOR, thought to be a precursor to microaspiration.
Highlights
Cystic fibrosis (CF) is a genetic condition caused by abnormalities in the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) gene
This indicated that microaspiration of refluxate in patients with CF might be a mechanism of injury linking gastrointestinal problems with lung disease [7]
Extra-oesophageal reflux (EOR) symptoms were present in six patients at baseline (RSI score N 13)
Summary
Cystic fibrosis (CF) is a genetic condition caused by abnormalities in the CF transmembrane conductance regulator (CFTR) gene. In pilot work we detected bile acids in the lower airways of 9 patients homozygous for F508del with advanced CF lung disease at the time of lung transplantation [5], and this potential source of injury persisted after lung transplantation [6] This indicated that microaspiration of refluxate in patients with CF might be a mechanism of injury linking gastrointestinal problems with lung disease [7]. Extra-oesophageal reflux (EOR) may lead to microaspiration in patients with cystic fibrosis (CF), a probable cause of deteriorating lung function. Treatment with ivacaftor was associated with a significant reduction of EOR symptoms (P b 0 ∙ 04 versus baseline) denoted by the reflux symptom index and Hull airway reflux questionnaire.
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