Childhood interstitial lung disease: A systematic review

Area of Science:

• Pediatric Pulmonology
• Rare Diseases
• Interstitial Lung Disease

Background:

• Childhood interstitial lung disease (chILD) encompasses rare, chronic, and complex pediatric lung disorders with varied pathologies.
• Existing research on chILD lacks systematic review, hindering comprehensive understanding.
• No prior studies have comprehensively reviewed chILD classification, epidemiology, treatment, outcomes, or familial/health service impact.

Purpose of the Study:

• To systematically review and describe childhood interstitial lung disease (chILD) classification systems.
• To outline the epidemiology, morbidity, treatments, and outcomes of chILD.
• To explore the impact of chILD on families and healthcare systems.

Main Methods:

• A systematic literature search was conducted in major biomedical databases up to December 2013.
• Included original studies on chILD, focusing on classification systems, epidemiology, and case series.
• Excluded single case studies to ensure a broader overview.

Main Results:

• 37 publications met the inclusion criteria for the review.
• Four distinct chILD classification systems have been proposed in the last decade.
• Estimated chILD incidence ranges from 0.13-16.2 cases/100,000 children/year, with a median mortality of 13% in developed countries.
• Morbidity and outcomes data were highly variable and inconsistently reported; corticosteroids and hydroxychloroquine were common treatments.
• The impact of chILD on families and health services remains unstudied.

Conclusions:

• The heterogeneity of chILD disorders and lack of standardized classification impede research consolidation.
• Consensus on chILD classification is crucial for improved diagnosis and research comparability.
• Active disease surveillance and international patient registries are essential for advancing chILD understanding and management.