Cystic fibrosis (CF) is a complex genetic chloride channelopathy, manifesting clinically as a
multisystem disorder. Patients with CF have significant morbidity and decreased life expectancy, primarily
due to respiratory disease, which is characterised by frequent pulmonary exacerbations and decline
in lung function. The pathogenesis of pulmonary disease in CF is explained in part by dehydration
of airway surface liquid with reduced mucociliary clearance leading to accumulation of thick inspissated
mucus in the airways. This results in airway obstruction, and provides a suitable environment for
pathogens to grow and colonize, giving rise to a cycle of recurrent airway inflammation in the CF lung.
Multiple therapeutic interventions in CF care are aimed at improving mucus clearance. These include
improved chest physiotherapy techniques, recombinant DNase, and more recently nebulised hypertonic
saline (HTS). Treatment with HTS has been shown to improve mucus clearance in CF and decrease exacerbations,
with subsequent improvements in quality of life, and lung function. While the mechanisms
by which HTS elicits these improvements are likely related to increase in mucociliary clearance, there is
increasing evidence to suggest that HTS also exerts significant anti-inflammatory and anti-microbial effects
in the CF lung. This review will describe these effects of HTS looking at the interaction between
HTS and neutrophils, the major inflammatory cell in the CF lung, and the effects of HTS on the antimicrobial
peptides found in the airways.
Keywords: Cystic fibrosis, hypertonic saline, mucociliary clearance, inflammation, neutrophils, lung function.
Rights & PermissionsPrintExport