Abstract
Background: Individuals with cystic fibrosis (CF) and fungal
airway infection may present with fungal bronchitis, allergic
bronchopulmonary aspergillosis (ABPA) or may appear unaffected despite
fungal detection. We sought to characterize people with CF with frequent
detection of fungi from airway samples and determine clinical outcomes.
Methods: This retrospective study included individuals with CF
with ≥ 4 lower airway cultures over a 2-year baseline period and ≥ 2
years of follow-up. We defined two groups: ≤ 1 positive fungus culture
(rare ) or ≥ 2 positive cultures during baseline
(frequent ). Clinical characteristics and outcomes were
determined.
Results: Between 2004-2016, 294 individuals met inclusion with
62% classified as rare and 38% as frequent fungi during baseline.
Median follow-up was 6 years (range 2-9 years). Aspergillusfumigatus was the most common fungal species detected.
Individuals with frequent fungi were older (13.7 vs. 11.7 yrs, p = 0.02)
and more likely to have Stenotrophomonas maltophilia (35% vs
17%, p < 0.001) at baseline, but did not differ in lung
function or ABPA diagnosis. During follow-up, those with frequent fungi
were more likely to have chronic P. aeruginosa and S.maltophilia . Individuals with ABPA and frequent fungi had the
highest rates of co-infection and co-morbidities, and a trend towards
more rapid lung function decline.
Discussion : Fungal infection in CF was associated with frequentP. aeruginosa and S. maltophilia co-infection even in
those without ABPA. Individuals with frequent fungi and ABPA had worse
outcomes, highlighting the potential contribution of fungi to CF
pulmonary disease.