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Early bronchiectasis linked to neutrophil elastase activity in lung fluid
Neutrophil elastase activity in bronchoalveolar lavage fluid in early life can lead to early bronchiectasis in children with cystic fibrosis, according to study results published in The New England Journal of Medicine.
“Bronchiectasis develops early in the course of cystic fibrosis, being detectable in infants as young as 10 weeks of age, and is persistent and progressive,” researchers wrote.
The study included 127 consecutive infants diagnosed with cystic fibrosis after newborn screening. Chest computed tomography and bronchoalveolar lavage (BAL) were performed when participants were in stable clinical condition at aged 3 months, 1, 2, and 3 years.
Researchers found the point prevalence of bronchiectasis at each visit increased from 29.3% at aged 3 months to 61.5% at aged 3 years.
Risk factors for bronchiectasis were presentation with meconium ileus (OR=3.17; 95% CI, 1.51-6.66); respiratory symptoms at the time of CT and BAL (OR=2.27; 95% CI, 1.24-4.14); free neutrophil elastase activity in BAL fluid (OR=3.02; 95% CI, 1.70-5.35); and gas trapping on expiratory CT (OR=2.05; 95% CI, 1.17-3.59).
“Free neutrophil elastase activity in the lung at 3 months of age was associated with increased odds of persistent bronchiectasis at 12 months and at 3 years of age,” researchers wrote. “This observation sets the stage for trials of treatments that target activated neutrophils or inhibit neutrophil elastase activity in order to prevent or delay the onset of bronchiectasis in patients with cystic fibrosis.”
Disclosure: The study was supported by the National Health and Medical Research Council of Australia and Cystic Fibrosis Foundation Therapeutics. See study for full list of disclosures.
