TY - JOUR T1 - Cured bronchi! Extending the use of nebulised hypertonic saline outside of cystic fibrosis? JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.00755-2018 VL - 51 IS - 5 SP - 1800755 AU - Clémence Martin AU - Lucile Regard AU - Pierre-Régis Burgel Y1 - 2018/05/01 UR - http://erj.ersjournals.com/content/51/5/1800755.abstract N2 - Plugging of the airways by excessive mucus is an important contributor to morbidity and mortality in chronic airway diseases (e.g. cystic fibrosis (CF), chronic obstructive pulmonary disease (COPD), bronchiectasis and primary ciliary dyskinesia (PCD)) [1]. Such findings are the basis for the major interest in drugs that reduce mucus content in airways by targeting mucus production [2] or by increasing clearance of mucus [3]. A single aerosol of hypertonic saline (HS) was shown to enhance clearance of airway secretions in humans >40 years ago [4], and a randomised control trial reported >10 years ago found that long-term nebulisation of HS resulted in decreased rates of pulmonary exacerbations in patients with CF [5]. However, the mechanisms underlying enhanced clearance of airway mucus and clinical benefits have not been fully elucidated [6]. In vitro studies supported the hypothesis that HS increased mucociliary clearance, as the administration of HS increased the volume of airway surface liquid (ASL) in cultured airway epithelial cells, an effect that was larger and more prolonged in cultures from patients with CF than in cultures of controls [7]. Based on these experimental and clinical results, HS is now used in many countries for the treatment of patients with CF [8]. However, there is only limited evidence that HS is effective in other chronic airway diseases. For example, the small number of studies comparing the effects of hypertonic versus isotonic saline in (non-CF) bronchiectasis yielded conflicting results [9, 10], and a recent 3-month crossover trial in patients with PCD found no effect on quality of life and lung function [11]. At first glance, it may be concluded that beneficial effects of HS in airway epithelium are limited to CF airways, with only limited effects in non-CF airways, due to altered ion transport in CF epithelium. However, it has been hypothesised that increase in the magnitude of effects of HS in CF versus normal epithelial cells is not related to cystic fibrosis transmembrane conductance regulator dysfunction itself, but to increased water retention [12] by the hyperconcentrated mucus that is found in CF airways [13, 14].Hypertonic saline may be effective not only in cystic fibrosis but also in other chronic airway diseases http://ow.ly/dioH30jGXoa ER -