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Updated: Jul 28, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Children's interstitial lung disease: Multidetector computed tomography patterns and correlations between imaging and
Päria Miraftabi1, Turkka Kirjavainen2, Janne S Suominen3
1HUS Medical Imaging Center, Radiology, University of Helsinki and Helsinki University Hospital, Finland.
Multidetector computed tomography (MDCT) patterns are infrequently identified in childhood interstitial lung disease (chILD). However, when a recognizable MDCT pattern is present, it accurately predicts the final diagnosis in children with chILD.
Area of Science:
- Pediatric Pulmonology
- Radiology
- Rare Diseases
Background:
- Childhood interstitial lung disease (chILD) encompasses diverse rare lung conditions unique to pediatric populations.
- Diagnosis relies on clinical findings, multidetector computed tomography (MDCT), genetic analysis, lung function tests, and lung biopsy.
- The diagnostic utility of MDCT pattern recognition in chILD remains underexplored.
Purpose of the Study:
- To investigate the prevalence of specific MDCT patterns in children with histologically confirmed interstitial lung disease.
- To assess the predictive value of identified MDCT patterns for the final chILD diagnosis.
Main Methods:
- Retrospective analysis of biopsy, MDCT, and clinical data from 90 pediatric patients (2004-2020).
- MDCT images were reviewed by blinded experts.
- Histological findings were classified according to established chILD categories.
Main Results:
- Sixty-three percent of the 90 included patients were male, with a median biopsy age of 1.3 years.
- Six distinct MDCT patterns were identified: neuroendocrine cell hyperplasia of infancy, organizing pneumonia, non-specific interstitial pneumonia, bronchiolitis obliterans, pulmonary alveolar proteinosis, and bronchopulmonary dysplasia.
- A recognizable MDCT pattern was present in 43% of patients; of these, 87% had a pattern predictive of the final diagnosis. In 57% of cases, no specific pattern was recognized.
Conclusions:
- Specific MDCT patterns are identified in less than half of childhood interstitial lung disease cases.
- When a distinct MDCT pattern is recognized in chILD, it demonstrates significant predictive value for the final diagnosis.
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