Evidence map›Paper›PMID 42287423›Full record

ArticlePediatric radiology2026

Utilizing a chest CT interstitial lung disease scoring system for commonly encountered pediatric rheumatologic diseases with associated interstitial lung disease with clinical correlation.

HaiThuy N Nguyen, Terry Robinson, Beverley Newman, Natalie A Mahieu, Paul G Thacker, Paul Iskander, Evan Zucker, Tzielan C Lee, Rajdeep Pooni, Joyce Hsu and 3 more

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Article in Pediatric radiology, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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1 · What the graph read from it

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2 · The registry

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3 · Its place in the literature

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4 · The record

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5 · Who and what money

Authors and funding

13 authors.

HaiThuy N NguyenDepartment of Radiology, Children's Hospital of Los Angeles, 4650 Sunset Blvd., Mailstop #81, Los Angeles, CA, 90027, USA. hainguyen@chla.usc.edu.
Terry RobinsonDepartment of Radiology, Children's Hospital of Los Angeles, 4650 Sunset Blvd., Mailstop #81, Los Angeles, CA, 90027, USA.
Beverley NewmanDepartment of Radiology, Divisions of Pediatric Radiology, Stanford Children's Hospital, Stanford, USA.
Natalie A MahieuDepartment of Radiology, Children's Hospital of Los Angeles, 4650 Sunset Blvd., Mailstop #81, Los Angeles, CA, 90027, USA.
Paul G ThackerDepartment of Radiology, Mayo Clinic, Rochester, USA.
Paul IskanderDepartment of Radiology, Divisions of Pediatric Radiology, Mattel Children's Hospital, Los Angeles, USA.
Evan ZuckerDepartment of Radiology, Divisions of Pediatric Radiology, Stanford Children's Hospital, Stanford, USA.
Tzielan C LeeDivision of Pediatric Rheumatology, Stanford University, Stanford, USA.
Rajdeep PooniDivision of Pediatric Rheumatology, Stanford University, Stanford, USA.
Joyce HsuDivision of Pediatric Rheumatology, Stanford University, Stanford, USA.
Lu TianDepartment of Biomedical Data Science, Stanford University, Stanford, USA.
Michal CidonDivision of Pediatric Rheumatology, Children's Hospital of Los Angeles, Los Angeles, USA.
Rex A MoatsDepartment of Radiology, Children's Hospital of Los Angeles, 4650 Sunset Blvd., Mailstop #81, Los Angeles, CA, 90027, USA.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundChest CT is useful for early detection and monitoring of rheumatologic-related interstitial lung disease (ILD) in children. However, interpretation is inconsistent and lacks standardization.

objectiveThis 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

methodsThis 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.

resultsMedian 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.

conclusionPRILD 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.

Indexed as

AdolescentsAutoimmune rheumatologic diseaseChest CT scansPediatricPediatric Rheumatologic Interstitial Lung Disease ScoreTotal Parenchymal ScoreYoung adults

Identifiers

PMID42287423

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