{Reference Type}: Journal Article
{Title}: Impact of lumacaftor/ivacaftor on the bacterial and fungal respiratory pathogens in cystic fibrosis: a prospective multicenter cohort study in Sweden.
{Author}: Al Shakirchi M;Sorjonen K;Hjelte L;Klingspor L;Bergman P;Ericson P;Svedberg M;Lindberg U;Hansen C;Monestrol I;
{Journal}: Ther Adv Respir Dis
{Volume}: 18
{Issue}: 0
{Year}: 2024 Jan-Dec
{Factor}: 5.158
{DOI}: 10.1177/17534666241254090
{Abstract}: <B>UNASSIGNED: </B>A significant decline in pulmonary exacerbation rates has been reported in CF patients homozygous for F508del treated with lumacaftor/ivacaftor. However, it is still unclear whether this reduction reflects a diminished microbiological burden.<BR><B>UNASSIGNED: </B>The aim of this study was to determine the impact of lumacaftor/ivacaftor on the bacterial and fungal burden.<BR><B>UNASSIGNED: </B>The study is a prospective multicenter cohort study including 132 CF patients homozygous for F508del treated with lumacaftor/ivacaftor.<BR><B>UNASSIGNED: </B>Clinical parameters as well as bacterial and fungal outcomes 1 year after initiation of lumacaftor/ivacaftor were compared to data from 2 years prior to initiation of the treatment. Changes in the slope of the outcomes before and after the onset of treatment were assessed.<BR><B>UNASSIGNED: </B>Lung function measured as ppFEV1 (p < 0.001), body mass index (BMI) in adults (p < 0.001), and BMI z-score in children (p = 0.007) were improved after initiation of lumacaftor/ivacaftor. In addition, the slope of the prevalence of Streptococcus pneumoniae (p = 0.007) and Stenotrophomonas maltophilia (p < 0.001) shifted from positive to negative, that is, became less prevalent, 1 year after treatment, while the slope for Candida albicans (p = 0.009), Penicillium spp (p = 0.026), and Scedosporium apiospermum (p < 0.001) shifted from negative to positive.<BR><B>UNASSIGNED: </B>The current study showed a significant improvement in clinical parameters and a reduction of some of CF respiratory microorganisms 1 year after starting with lumacaftor/ivacaftor. However, no significant changes were observed for Pseudomonas aeruginosa, Staphylococcus aureus, or Aspergillus fumigatus, key pathogens in the CF context.