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Published on: August 9, 2024
Longitudinal Magnetic Resonance Imaging of Changes in Lung Morphology and Perfusion in Children with Cystic Fibrosis
Mark O Wielpütz1,2,3, Mirjam Stahl4,5,6, Simon M F Triphan1,2,3
1Department of Diagnostic and Interventional Radiology.
Insights
Lung changes in cystic fibrosis (CF) worsen throughout childhood, with significant increases in bronchiectasis, mucus plugging, and perfusion abnormalities observed via MRI. This study provides age-specific references for CF lung disease severity in children.
Area of Science:
- Pediatric radiology
- Pulmonology
- Medical imaging
Background:
- Longitudinal imaging data on lung changes in cystic fibrosis (CF) during childhood is limited.
- Understanding disease progression from infancy to adolescence is crucial for effective management.
Purpose of the Study:
- To longitudinally assess changes in lung morphology and perfusion in pediatric patients with CF using chest MRI.
- To establish age-specific reference percentiles for CF lung disease severity.
Main Methods:
- 1112 annual chest MRI scans were analyzed in 226 CF patients aged 0-18 years.
- A validated MRI scoring system was used to quantify lung morphology and perfusion abnormalities.
- Prevalence and severity of bronchiectasis, wall thickening, and mucus plugging were assessed.
Main Results:
- Global MRI scores significantly increased from infancy to adolescence (5.5 to 17.9).
- Bronchiectasis and mucus plugging prevalence and severity increased with age, reaching near 100% and significant subscore increases, respectively.
- Perfusion abnormalities rose from 44.4% in infancy to approximately 90% by preschool age, with scores increasing significantly.
Conclusions:
- This is the first study to longitudinally track lung morphology and perfusion changes in pediatric CF patients using MRI.
- The findings provide crucial age-specific reference data for CF lung disease severity.
- MRI is proposed as a valuable endpoint for clinical trials in pediatric CF.
Abstract:
Rationale: The progression of lung changes in cystic fibrosis (CF) from infancy through adolescence remains poorly understood as a result of limited longitudinal imaging data. Objectives: To assess changes in lung morphology and perfusion in children with CF through the pediatric age range by longitudinal chest magnetic resonance imaging (MRI). Methods: 1,112 annual chest MRI scans were performed in 226 patients with CF aged 0-18 years. MRI was assessed using a validated MRI scoring system. Results: The MRI global score continuously increased from 5.5 ± 4.6 at infancy (0 yr) to 17.9 ± 8.5 at adolescence (12-18 yr), and the MRI morphology score increased from 5.0 ± 3.9 to 12.3 ± 6.1 (P < 0.001). Bronchiectasis/wall thickening prevalence increased from 89.1% at infancy to approximately 100% at preschool age (1-5 yr), and the subscore increased from 3.1 ± 1.9 at infancy to 6.6 ± 2.1 at adolescence (P < 0.001). Mucus plugging prevalence increased from 55.4% at infancy to 83.0% at adolescence, and the subscore increased from 1.2 ± 1.6 to 3.7 ± 2.6 in the same period (P < 0.001). Perfusion abnormalities were found in 44.4% at infancy, and increased to approximately 90% at preschool age (P < 0.001). The MRI perfusion score increased from 1.1 ± 1.6 at infancy to 5.6 ± 3.0 at adolescence (P < 0.001). Chronic Pseudomonas aeruginosa infection was associated with higher MRI scores at school age (6-11 yr; P < 0.05-0.001). Conclusions: This is the first study to assess longitudinal changes in lung morphology and perfusion in CF throughout the pediatric age range, providing percentiles as age-specific references for lung disease severity. Our data may facilitate the use of MRI as an endpoint in clinical trials in children with CF. Clinical trial registered with www.clinicaltrials.gov (NCT00760071 and NCT02270476).
