Prevalence of gastroesophageal reflux in cystic fibrosis and implications for lung disease.

Gastroesophageal reflux (GER) is common in patients with cystic fibrosis (CF) and is often regarded as playing a role in the pathogenesis of CF lung disease. Individuals with CF have many predisposing factors to the development of GER, with a reported prevalence ranging from 35 to 81%. Several studies have suggested that patients with CF who have coexisting GER have more severe lung disease with lower pulmonary function and increased numbers of respiratory exacerbations. Furthermore, GER may alter the respiratory microbiology in CF. Both the acid and nonacid components of GER may have an effect on lung disease. More than 50% of U.S. patients with CF were being treated with proton pump inhibitors in 2012; however, data regarding safety and efficacy of these agents in CF are lacking. Pharmacologic and surgical treatment of GER may improve respiratory morbidity, although prospective controlled studies have not been performed. Given the lack of evidence-based guidelines for evaluation, diagnosis, and treatment of GER in CF, initiation of treatment for symptomatic GER should be based on standard guidelines for the general population. Because there is no clear evidence that GER leads to worse respiratory outcomes in CF or that treatment of GER improves pulmonary outcomes, invasive testing for GER in patients without reflux symptoms is not warranted. Further studies to determine the role of GER in CF lung disease and the risks and benefits of surgical and pharmacologic therapy for GER are warranted.