Proteomic profiling identifies novel circulating markers associated with bronchiectasis in cystic fibrosis

Area of Science:

• Pulmonary Medicine
• Proteomics
• Pediatric Research

Background:

• Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs, primarily the lungs.
• Bronchiectasis, an irreversible airway dilation, is a common complication in CF, leading to progressive lung damage.
• Early detection and monitoring of bronchiectasis are crucial for managing CF lung disease.

Purpose of the Study:

• To evaluate changes in bronchiectasis over one year in children with CF.
• To identify blood proteins associated with bronchiectasis in this pediatric population.

Main Methods:

• A pilot study involving 26 children with CF who underwent chest CT scans and blood collection during clinical stability.
• Analysis of 1129 plasma proteins using the SOMAscan proteomics platform.
• Measurement of bronchiectasis and airway count changes on CT scans taken one year apart, correlated with protein levels.

Main Results:

• Bronchiectasis and airway count showed significant changes over one year in the studied children.
• Proteins linked to inflammation and extracellular matrix degradation were associated with both cross-sectional and longitudinal structural lung changes.
• Circulating protein levels demonstrated stronger correlations with imaging outcomes than age or spirometry.

Conclusions:

• Novel blood proteins associated with bronchiectasis were identified in children with CF using the SOMAscan platform.
• These identified proteins show potential as clinically useful biomarkers for monitoring bronchiectasis in pediatric CF patients.
• The findings suggest proteomics can offer valuable insights into CF lung disease progression.