COMBATing airway inflammation in infants with cystic fibrosis

Abstract

Cystic fibrosis lung disease is characterised by a progressive cycle of mucus obstruction, airway infection, and inflammation. The Australian Respiratory Early Surveillance Team for Cystic Fibrosis (AREST CF) 1 Sly PD Gangell CL Chen L et al. Risk factors for bronchiectasis in children with cystic fibrosis. N Engl J Med. 2013; 368: 1963-1970 Crossref PubMed Scopus (401) Google Scholar intensive surveillance programme has provided strong evidence that this process starts early in infancy, before respiratory symptoms. In a landmark study, the team reported that free neutrophil elastase in bronchoalveolar lavage done in infancy predicts the subsequent development of bronchiectasis by age 3 years, rendering airway inflammation a key target to modulate disease progression early in life. 1 Sly PD Gangell CL Chen L et al. Risk factors for bronchiectasis in children with cystic fibrosis. N Engl J Med. 2013; 368: 1963-1970 Crossref PubMed Scopus (401) Google Scholar The effect of azithromycin on structural lung disease in infants with cystic fibrosis (COMBAT CF): a phase 3, randomised, double-blind, placebo-controlled clinical trialAzithromycin treatment from diagnosis of cystic fibrosis did not reduce the extent of structural lung disease at 36 months of age; however, it did reduce airway inflammation, morbidity including pulmonary exacerbations in the first year of life and hospitalisations, and improved some clinical outcomes associated with cystic fibrosis lung disease. Therefore we suggest thrice-weekly azithromycin is a strategy that could be considered for the routine early management of paediatric patients with cystic fibrosis. Full-Text PDF