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Published on: January 19, 2022
Airway Inflammation in Children with Primary Ciliary Dyskinesia
Scott D Sagel1, Oren Kupfer1, Brandie D Wagner2
1Department of Pediatrics, Children's Hospital Colorado, University of Colorado Anschutz Medical Campus, Aurora, Colorado.
Insights
In children with primary ciliary dyskinesia (PCD), higher sputum inflammation markers correlate with worse lung function and structural damage. These inflammatory measurements may serve as useful biomarkers for disease progression in pediatric PCD patients.
Area of Science:
- Pediatric Pulmonology
- Respiratory Medicine
- Immunology
Background:
- Airway inflammation's role in primary ciliary dyskinesia (PCD) pathogenesis in children is not well understood.
- Investigating inflammation markers in pediatric PCD is crucial for understanding disease progression and identifying therapeutic targets.
Purpose of the Study:
- To examine the associations between sputum inflammation markers, age, lung function, bronchiectasis, airway infection, and ultrastructural defects in children with PCD.
- To determine if sputum inflammatory measurements can serve as biomarkers in pediatric PCD.
Main Methods:
- Collected spontaneously expectorated sputum from 77 clinically stable children and adolescents (ages 6+) with PCD in a multicenter observational study.
- Correlated sputum protease and inflammatory cytokine concentrations with age, lung function (FEV1% predicted), and chest CT measures of structural lung disease.
- Compared inflammatory marker concentrations between different ultrastructural defect categories and between those with and without detectable bacterial infection.
Main Results:
- Elevated sputum neutrophil elastase, IL-1β, IL-8, and TNF-α concentrations positively correlated with age, bronchiectasis extent, and overall structural lung disease on CT.
- These inflammatory markers negatively correlated with lung function (FEV1% predicted).
- Higher concentrations of sputum neutrophil elastase, IL-1β, and TNF-α were found in children with detectable bacterial pathogens.
Conclusions:
- In pediatric PCD, increased sputum proteases and cytokines are linked to diminished lung function and structural lung damage.
- Sputum inflammatory measurements show potential as valuable biomarkers for assessing disease status and progression in children with PCD.
Abstract:
Rationale: The role of airway inflammation in disease pathogenesis in children with primary ciliary dyskinesia (PCD) is poorly understood. Objectives: We investigated relationships between sputum inflammation measurements, age, lung function, bronchiectasis, airway infection, and ultrastructural defects in children with PCD. Methods: Spontaneously expectorated sputum was collected from clinically stable children and adolescents with PCD ages 6 years and older participating in a multicenter, observational study. Sputum protease and inflammatory cytokine concentrations were correlated with age, lung function, and chest computed tomography measures of structural lung disease, whereas differences in concentrations were compared between ultrastructural defect categories and between those with and without detectable bacterial infection. Results: Sputum from 77 children with PCD (39 females [51%]; mean [standard deviation] age, 13.9 [4.9] yr; mean [standard deviation] forced expiratory volume in 1 second [FEV1]% predicted, 80.8 [20.5]) was analyzed. Sputum inflammatory marker measurements, including neutrophil elastase activity, IL-1β (interleukin-1β), IL-8, and TNF-α (tumor necrosis factor α) concentrations, correlated positively with age, percentage of bronchiectasis, and percentage of total structural lung disease on computed tomography, and negatively with lung function. Correlations between neutrophil elastase concentrations and FEV1% predicted and percentage of bronchiectasis were -0.32 (95% confidence interval, -0.51 to -0.10) and 0.46 (0.14 to 0.69), respectively. Sputum neutrophil elastase, IL-1β, and TNF-α concentrations were higher in those with detectable bacterial pathogens. Participants with absent inner dynein arm and microtubular disorganization had similar inflammatory profiles compared with participants with outer dynein arm defects. Conclusions: In this multicenter pediatric PCD cohort, elevated concentrations of sputum proteases and cytokines were associated with impaired lung function and structural damage as determined by chest computed tomography, suggesting that sputum inflammatory measurements could serve as biomarkers in PCD.
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