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Updated: Jun 6, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Imaging Spectrum of Childhood Interstitial Lung Diseases: Focus on Disorders Not Specific to Infancy
Orito Ikeda1, Yuko Tsujioka1, Gen Nishimura2
1Department of Radiology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, Japan 160-8582.
Insights
Childhood interstitial lung disease (chILD) is a group of rare conditions. High-resolution CT imaging is crucial for diagnosing chILD by integrating various findings for accurate identification.
Area of Science:
- Pediatric Pulmonology
- Radiology
- Rare Diseases
Background:
- Childhood interstitial lung disease (chILD) encompasses diverse rare disorders previously lacking clear classification.
- A new classification system by the chILD Research Network provides a systematic approach to identifying and categorizing these conditions.
- chILDs are collectively frequent in pediatric practice, necessitating prompt diagnosis for improved outcomes.
Purpose of the Study:
- To review current knowledge on imaging findings in childhood interstitial lung disease.
- To highlight the role of high-resolution CT in diagnosing chILD.
- To emphasize the integration of clinical, radiologic, pathologic, and genetic data for accurate diagnosis.
Main Methods:
- Review of current scientific literature on chILD imaging.
- Analysis of imaging findings in acute and chronic phases of chILD.
- Integration of imaging patterns with clinical, pathological, and genetic data.
Main Results:
- High-resolution CT is central to diagnosing chILD due to nonspecific clinical symptoms.
- Acute-phase imaging may show ground-glass opacities and crazy-paving patterns.
- Chronic-phase findings include reticulation, cysts, honeycombing, and mosaic attenuation, all requiring integration for diagnosis.
Conclusions:
- Accurate diagnosis of chILD requires a multidisciplinary approach integrating diverse findings.
- High-resolution CT imaging patterns, when meticulously analyzed, are key to identifying specific chILD diagnoses.
- Early and precise diagnosis of chILD through integrated imaging improves patient outcomes.
Abstract:
Childhood interstitial lung disease (chILD) comprises a diverse group of rare disorders, which were previously poorly categorized on clinical, histologic, and pathogenic grounds. The classification of chILD introduced by the chILD Research Network has led to a systematic approach to identifying and categorizing these rare conditions. A multidisciplinary approach integrating clinical, radiologic, pathologic, and genetic findings enables accurate diagnosis in children with "pediatric interstitial pneumonia of uncertain cause." It has become apparent that chILDs are collectively frequent in pediatric practice, and prompt diagnosis can improve the outcomes of affected children. Since the clinical symptoms of chILDs are nonspecific, imaging using high-resolution CT plays a central role in the diagnostic approach to this diverse group of diseases. In most disorders, cellular infiltration and abnormal exudates commonly cause acute-phase imaging findings, such as centrilobular ground-glass opacities that are multifocal, geographic, and diffuse, as well as a crazy-paving appearance. Persistent inflammatory reactions lead to chronic-phase findings, including reticulation, lung cysts, and honeycombing with traction bronchiectasis. Small-airway compromise causes air trapping and a mosaic attenuation pattern. These imaging findings are individually nonspecific; however, meticulous integration of the diverse imaging findings allows a specific diagnosis to be reached. The authors review the current knowledge on the imaging findings associated with chILDs based on recent investigations of these conditions. ©RSNA, 2026 Supplemental material is available for this article.
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