Abstract
Bacterial and fungal infections are hallmarks of cystic fibrosis (CF) lung disease. In the era of long-term inhaled antibiotics and increasing CF patient survival, new “emerging” pathogens are detected in CF airways, yet their pathophysiological disease relevance remains largely controversial and incompletely defined. As a response to chronic microbial triggers, innate immune cells, particularly neutrophils, are continuously recruited into CF airways where they combat pathogens but also cause tissue injury through release of oxidants and proteases. The coordinated interplay between host immune cell activation and pathogens is essential for the outcome of CF lung disease. Here, we provide a concise overview and update on host-pathogen interactions in CF lung disease.
Highlights
Bacterial and fungal infections are hallmarks of cystic fibrosis (CF) lung disease
CF lung disease Lung disease determines the morbidity and mortality of patients with cystic fibrosis (CF), a lethal monogenetic disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene [21]
Microbial airway colonization in CF lung disease CF airways are mainly colonized by specific bacteria and fungi [28]
Summary
In the era of long-term inhaled antibiotics and increasing CF patient survival, new “emerging” pathogens are detected in CF airways, yet their pathophysiological disease relevance remains largely controversial and incompletely defined. As a response to chronic microbial triggers, innate immune cells, neutrophils, are continuously recruited into CF airways where they combat pathogens and cause tissue injury through release of oxidants and proteases. The underlying host-pathogen interaction mechanisms regulating the CF-characteristic microbial “switch” from S. aureus and H. influenzae to P. aeruginosa remain, controversial and incompletely understood, but probably involve pathogen-derived factors, such as pyocyanin and host-derived immune factors as well as environmental influences. When innate immune cells are in physical contact with pathogens, several factors decide which anti-microbial defense mechanisms are employed; phagocytotic uptake is the most rapid and principal effector function against smaller bacteria and fungi, after antibodyand/or complement-mediated opsonisation [20]. Studies involved a dysregulated ceramide homeostasis/turnover in CF lung disease by showing that ceramide accumulates in CF airways and mediates inflammation, cell death, and infection susceptibility [29]
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
