Connective tissue disease-associated lung disease in children
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Updated: Jun 24, 2025

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Andrew H Schapiro1,2, Cara E Morin3,4, Kathryn A Wikenheiser-Brokamp5,6,7,8
1Department of Radiology, Cincinnati Children's Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH, 45229, USA. andrew.schapiro@cchmc.org.
Insights
Connective tissue diseases can impact children's lungs, causing serious illness. Recognizing specific radiologic-pathologic patterns on CT scans aids in early diagnosis and management of pediatric lung disease.
Area of Science:
- Pediatric Rheumatology
- Pulmonary Medicine
- Pediatric Radiology
Background:
- Connective tissue diseases (CTDs) are autoimmune disorders affecting multiple organ systems.
- Lung involvement in pediatric CTDs significantly increases morbidity and mortality.
- CTD-associated lung disease in children presents with distinct radiologic-pathologic patterns.
Purpose of the Study:
- To review key clinical, histopathologic, and CT features of pediatric CTD-associated lung disease.
- To emphasize radiologic-pathologic correlation for improved diagnostic accuracy.
- To enhance the role of pediatric radiologists in managing these complex cases.
Main Methods:
- Review of clinical presentations.
- Analysis of histopathologic findings.
- Correlation with computed tomography (CT) imaging patterns.
Main Results:
- Specific radiologic-pathologic patterns are associated with particular CTDs in children.
- Understanding these patterns aids in differential diagnosis.
- CT is crucial for identifying and characterizing lung disease in pediatric CTD.
Conclusions:
- Radiologic-pathologic correlation is essential for diagnosing CTD-associated lung disease in children.
- Accurate CT interpretation empowers pediatric radiologists to improve patient care.
- Early recognition of lung disease patterns facilitates timely intervention in pediatric CTD.
Abstract:
Connective tissue diseases are a heterogeneous group of autoimmune diseases that can affect a variety of organ systems. Lung parenchymal involvement is an important contributor to morbidity and mortality in children with connective tissue disease. Connective tissue disease-associated lung disease in children often manifests as one of several radiologic-pathologic patterns of disease, with certain patterns having a propensity to occur in association with certain connective tissue diseases. In this article, key clinical, histopathologic, and computed tomography (CT) features of typical patterns of connective tissue disease-associated lung disease in children are reviewed, with an emphasis on radiologic-pathologic correlation, to improve recognition of these patterns of lung disease at CT and to empower the pediatric radiologist to more fully contribute to the care of pediatric patients with these conditions.
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