Clinical characteristics of people with cystic fibrosis and frequent
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).
Aspergillus fumigatus 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 frequent P.
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