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Updated: Jun 16, 2026
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Utilizing a chest CT interstitial lung disease scoring system for commonly encountered pediatric rheumatologic
HaiThuy N Nguyen1, Terry Robinson2, Beverley Newman3
1Department of Radiology, Children's Hospital of Los Angeles, 4650 Sunset Blvd., Mailstop #81, Los Angeles, CA, 90027, USA. hainguyen@chla.usc.edu.
Insights
A new scoring system for pediatric rheumatologic interstitial lung disease (ILD) shows promise in evaluating chest CT scans. Further refinement and multicenter collaboration are needed to improve its reliability for diagnosing childhood ILD.
Area of Science:
- Pediatric Radiology
- Rheumatology
- Pulmonary Medicine
Background:
- Chest CT is crucial for detecting and monitoring interstitial lung disease (ILD) in children with rheumatologic conditions.
- Current interpretation of these CT scans lacks standardization, leading to inconsistent assessments.
Purpose of the Study:
- To systematically assess rheumatologic-related ILD in pediatric patients using existing CT scoring systems.
- To correlate CT findings with clinical data in children diagnosed with rheumatologic ILD.
Main Methods:
- A retrospective study analyzed chest CT scans of patients aged 0-21 years with rheumatologic-related ILD.
- A novel Pediatric Rheumatologic Interstitial Lung Disease (PRILD) scoring system was developed and applied by blinded pediatric radiologists.
- Clinical data and pulmonary function tests were correlated with PRILD scores.
Main Results:
- The PRILD scoring system identified significantly higher scores in patients with active rheumatologic disease compared to controls.
- Interrater agreement for the PRILD scoring system was poor (ICC=0.41), indicating variability in interpretation.
- Weak negative correlations were observed between CT scores and pulmonary function tests.
Conclusions:
- The PRILD scoring system offers a structured approach to evaluating thoracic involvement in pediatric rheumatologic ILD.
- Improvements in interrater agreement through further refinement and education are necessary.
- Multicenter collaboration is essential for validating the PRILD scoring system due to the rarity of these conditions.
Background:
Chest CT is useful for early detection and monitoring of rheumatologic-related interstitial lung disease (ILD) in children. However, interpretation is inconsistent and lacks standardization.
Objective:
This study employs prior disease-specific CT scoring systems to systematically assess most rheumatologic diseases with ILD in pediatric patients and correlate scores with clinical findings.
Materials And Methods:
This retrospective study included patients (ages 0-21 years) with rheumatologic-related ILD and chest CT. Two pediatric radiologists determined CT quality and separated patients into disease and disease control groups. Three different blinded pediatric radiologists scored CT scans using this Pediatric Rheumatologic Interstitial Lung Disease (PRILD) scoring system, comprising of parenchymal, nodule/mass/cavitary/cyst, and pleural/pericardial components (total score, 125). Clinical data were extracted from medical records. Non-parametric tests were used for group comparisons, intraclass correlation coefficient (ICC) for interobserver agreement, and Spearman's correlation for CT and pulmonary function relationships.
Results:
Median age was 13.8 years and 84% were female. The disease group had statistically significant higher median total PRILD scores than the disease control group (7.3 vs. 1.7, P<0.001) and a higher percent active rheumatologic disease (93% vs 78%, P=0.039). There were no statistically significant differences between the two groups regarding age, sex, rheumatologic disease type, disease duration, or pulmonary symptoms. Interrater agreement was poor; ICC was 0.41. When both groups were combined, there were weak negative correlations with pulmonary function tests.
Conclusion:
PRILD scoring system aids in systematic evaluation of thoracic involvement in rheumatologic-related ILD. However, further refinement and education are needed to improve interrater agreement, and given the rare nature of these diseases, a multicenter collaboration is necessary for validation of this CT scoring system.
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