Abstract
In response to recurrent infection in cystic fibrosis (CF), powerful innate immune signals trigger polymorphonuclear neutrophil recruitment into the airway lumen. Exaggerated neutrophil proteolytic activity results in sustained inflammation and scarring of the airways. Consequently, neutrophils and their secretions are reliable clinical biomarkers of lung disease progression. As neutrophils are required to clear infection and yet a direct cause of airway damage, modulating adverse neutrophil activity while preserving their pathogen fighting function remains a key area of CF research. The factors that drive their pathological behavior are still under investigation, especially in early disease when aberrant neutrophil behavior first becomes evident. Here we examine the latest findings of neutrophils in pediatric CF lung disease and proposed mechanisms of their pathogenicity. Highlighted in this review are current and emerging experimental methods for assessing CF mucosal immunity and human neutrophil function in the laboratory.
Highlights
Polymorphonuclear neutrophils are the most abundant immune cells in human blood and act as first responders to sites of infection
New data from modern clinical studies are changing the view that neutrophils are a fixed population and are revealing a spectrum of functional phenotypes neutrophils employ to address the variety of pathogenic scenarios they encounter [4]
We present new insights, challenges, and Systems for Studying Neutrophil Plasticity considerations for researchers studying neutrophils in early cystic fibrosis (CF) lung disease
Summary
Polymorphonuclear neutrophils are the most abundant immune cells in human blood and act as first responders to sites of infection. Their function is a key component of host defense against invading pathogens. New data from modern clinical studies are changing the view that neutrophils are a fixed population and are revealing a spectrum of functional phenotypes neutrophils employ to address the variety of pathogenic scenarios they encounter [4]. Understanding neutrophil phenotypes and mechanisms in early CF disease, when the airway environment is less complex and more responsive to intervention, will require researchers to revisit or adapt many models of CF. Systems for Studying Neutrophil Plasticity considerations for researchers studying neutrophils in early CF lung disease
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