Key Cystic Fibrosis Pathogens Reduced With Ivacaftor Use

For patients with cystic fibrosis (CF), ivacaftor use is associated with a reduction in pathogens, including Pseudomonas aeruginosa, according to a study published online in the Annals of the American Thoracic Society.

Freddy J. Frost, B.M.B.S., from Liverpool Heart & Chest Hospital National Health Service Foundation Trust in the United Kingdom, and colleagues conducted a retrospective cohort study using data from the U.K. CF Registry from 2011 to 2016 to examine changes in respiratory microbiology associated with long-term ivacaftor use. Data were included for 276 patients with CF who used ivacaftor and 5,296 in the nonivacaftor comparator group.

The researchers observed an early and sustained reduction in P. aeruginosa rates in association with ivacaftor use (adjusted prevalence ratio, 0.68) via a combination of elevated clearance in those with infection (37.9 versus 22.8 percent for ivacaftor versus nonivacaftor cohorts) and reduced acquisition among those without infection (36.6 versus 48.6 percent). The reduction of P. aeruginosa infection prevalence was independent of reduced sampling in the ivacaftor cohort. Ivacaftor use also correlated with reduced Staphylococcus aureus and Aspergillus species prevalence but was not associated with a reduction in Burkholderia cepacia complex.

"Our findings of reduced CF pathogens are relevant clinically given the morbidity associated with chronic infection by these species, and imply there may be potential to safely reduce the treatment burden in some patients," the authors write.

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