Paediatric interstitial lung disease: classification and definitions

Area of Science:

• Pediatric Pulmonology
• Histopathology
• Rare Lung Diseases

Background:

• Classifications for interstitial (diffuse) lung disease (ILD) in children have evolved significantly.
• Advances in understanding new entities, biology, and prognostic significance of histologic patterns have driven these revisions.
• The European Respiratory Society Task Force and North American Children's Interstitial Lung Disease Group have made key contributions.

Purpose of the Study:

• To review recent revisions in pediatric interstitial lung disease classifications.
• To summarize a clinicopathologic classification of paediatric diffuse lung disease.
• To present clinical characteristics and histologic definitions for specific entities.

Main Methods:

• Review of contributions from the European Respiratory Society Task Force on Interstitial Lung Disease in Children.
• Review of contributions from the North American Children's Interstitial Lung Disease Group.
• Summarization of a clinicopathologic classification for paediatric diffuse lung disease.

Main Results:

• A comprehensive clinicopathologic classification of paediatric diffuse lung disease is presented.
• Clinical characteristics and histologic definitions are provided for selected entities including acinar dysgenesis, congenital alveolar dysplasia, and neuroendocrine cell hyperplasia of infancy.
• The classification encompasses a range of conditions such as surfactant dysfunction disorders, obliterative bronchiolitis, and immunologic disorders.

Conclusions:

• Uniform application of revised diagnostic terminology is crucial for future research.
• Standardized terminology will enable more meaningful comparisons of patient populations.
• This will facilitate improved radiologic-pathologic correlation and the development of disease-specific therapeutic strategies for pediatric diffuse lung diseases.