Pulmonary exacerbations in CF patients with early lung disease

J Cyst Fibros. 2014 Jan;13(1):74-9. doi: 10.1016/j.jcf.2013.07.006. Epub 2013 Sep 10.


Background: Current definitions of pulmonary exacerbation (PE) in cystic fibrosis are based on studies in participants with significant lung disease and may not reflect the spectrum of findings observed in younger patients with early lung disease.

Methods: We used data from a recent trial assessing the efficacy of azithromycin in children to study signs and symptoms associated with PEs and related changes in lung function and weight.

Results: While increased cough was present in all PEs, acute weight loss and reduction in oxygen saturation were not observed. Changes in lung function did not differ between subjects who did experience a PE and those who were exacerbation-free.

Conclusions: Cough was the predominant symptom in CF patients with early lung disease experiencing a PE. There was no significant difference in mean 6-month change in lung function or weight among subjects with one or more exacerbations and those without an exacerbation.

Keywords: Azithromycin; Chronic; Cystic fibrosis; Infection; Lung function; Mild; Pseudomonas aeruginosa; Pulmonary exacerbation; Weight.

Publication types

  • Multicenter Study
  • Randomized Controlled Trial

MeSH terms

  • Adolescent
  • Adult
  • Anti-Bacterial Agents / therapeutic use
  • Azithromycin / therapeutic use*
  • Child
  • Cough / drug therapy
  • Cough / physiopathology
  • Cystic Fibrosis / drug therapy*
  • Cystic Fibrosis / pathology
  • Cystic Fibrosis / physiopathology*
  • Disease Progression
  • Double-Blind Method
  • Female
  • Humans
  • Lung / pathology
  • Lung / physiopathology
  • Lung Diseases / drug therapy*
  • Lung Diseases / pathology
  • Lung Diseases / physiopathology*
  • Male
  • Middle Aged
  • Respiratory Function Tests
  • Respiratory Tract Infections / drug therapy
  • Respiratory Tract Infections / pathology
  • Respiratory Tract Infections / physiopathology


  • Anti-Bacterial Agents
  • Azithromycin