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Updated: Jun 11, 2025
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Children interstitial lung disease: Assessment and management
Antonio Moreno-Galdo1,2, Ignacio Iglesias-Serrano1, Sandra Rovira-Amigo1,2
1Section of Pediatric Allergy and Pulmonology, Department of Pediatrics, Vall d'Hebron Hospital Universitari, Vall d'Hebron Barcelona Hospital Campus, Universitat Autònoma de Barcelona, Barcelona, Spain.
Children interstitial lung disease (chILD) encompasses over 200 rare conditions, with higher prevalence than previously thought. Early diagnosis and a multidisciplinary approach are crucial for managing these complex pediatric lung diseases.
Area of Science:
- Pediatric Pulmonology
- Rare Diseases
- Genetics
Background:
- Children interstitial lung disease (chILD) comprises over 200 rare, heterogeneous conditions.
- Prevalence is higher than previously recognized, particularly in children under two years old.
- Early-onset chILD is often genetic or linked to lung development disorders.
Purpose of the Study:
- To highlight the heterogeneity and prevalence of pediatric interstitial lung diseases.
- To emphasize the importance of early diagnosis and multidisciplinary care.
- To discuss diagnostic approaches and the evolving landscape of chILD treatments.
Main Methods:
- Review of current understanding of chILD incidence and prevalence.
- Description of diagnostic workup including imaging, bronchoscopy, and genetic testing.
- Discussion of the genetic basis and undefined causes of chILD.
Main Results:
- chILD incidence is ~8.2/million, prevalence ~46.4/million, higher than previously estimated.
- 40-60% of cases occur in children under 2 years.
- Genetic causes identified in 10-20%, with 12% remaining undefined (undefined chILD).
Conclusions:
- A multidisciplinary expert center approach is essential for chILD diagnosis and management.
- Comprehensive diagnostic testing, including genetic studies, is vital.
- While specific treatments are limited, understanding pathophysiology aids new therapy development.
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