Abstract
Fungi are frequently recovered from lower airway samples from people with cystic fibrosis (CF), yet the role of fungi in the progression of lung disease is debated. Recent studies suggest worsening clinical outcomes associated with airway fungal detection, although most studies to date are retrospective or observational. The presence of fungi can elicit a T helper cell type 2 (Th-2) mediated inflammatory reaction known as allergic bronchopulmonary aspergillosis (ABPA), particularly in those with a genetic atopic predisposition. In this review, we discuss the epidemiology of fungal infections in people with CF, risk factors associated with development of fungal infections, and microbiologic approaches for isolation and identification of fungi. We review the spectrum of fungal disease presentations, clinical outcomes after isolation of fungi from airway samples, and the importance of considering airway co-infections. Finally, we discuss the association between fungi and airway inflammation highlighting gaps in knowledge and future research questions that may further elucidate the role of fungus in lung disease progression.
Highlights
Cystic Fibrosis (CF) is one of the most common inherited conditions, resulting in early morbidity and shortened life expectancy [1]
Pseudomonas aeruginosa, Staphylococcus aureus, and other gram-negative bacteria are frequently detected from airway samples and chronic infection with these pathogens associated with worse outcomes [7,8,9,10,11,12]
A. fumigatus and other Aspergillus species are the most frequently detected fungi with reported prevalence ranging from 10–50%, there is a variety in patient populations and clinical laboratory practices that impacts detection rates as reported by multiple reviews and studies [15,16,25,26]
Summary
Cystic Fibrosis (CF) is one of the most common inherited conditions, resulting in early morbidity and shortened life expectancy [1]. Individuals with CF are prone to persistent airway infections due to dehydrated mucus that accumulates in the airways [3,4] This abnormal mucus creates a protein rich environment for organisms to grow, leading to chronic infection and resulting in inflammatory response [5,6]. This in turn leads to airway obstruction, remodeling and bronchiectasis, causing a progressive decline in lung function. Pseudomonas aeruginosa, Staphylococcus aureus, and other gram-negative bacteria are frequently detected from airway samples and chronic infection with these pathogens associated with worse outcomes (e.g., lower lung function, increased frequency of pulmonary exacerbation) [7,8,9,10,11,12].
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