Pulmonary function testing in children's interstitial lung disease

Area of Science:

• Pediatric Pulmonology
• Respiratory Medicine
• Interstitial Lung Diseases

Background:

• Pulmonary function tests (PFTs) are established for airway diseases but their role in children's interstitial lung disease (chILD) remains unclear.
• The broad spectrum of chILD pathologies and heterogeneous patient population complicate PFT interpretation.
• Despite widespread use in adult interstitial lung disease, PFT utility in pediatric populations requires further investigation.

Purpose of the Study:

• To review existing literature on PFT use in chILD.
• To identify knowledge gaps regarding PFTs in chILD diagnosis and management.
• To propose future research directions for optimizing PFT application in chILD.

Main Methods:

• A literature review was conducted by the COST/Enter chILD working group.
• The review focused on spirometry, whole-body plethysmography, infant/pre-school PFTs, diffusing capacity, multiple breath washout, and cardiopulmonary exercise tests.
• Studies published over the past three decades were analyzed.

Main Results:

• A limited number of studies on PFTs in chILD were identified over the last 30 years.
• Most published studies were descriptive, with pulmonary function not being the primary outcome.
• Current data is insufficient to establish definitive PFT protocols for chILD.

Conclusions:

• PFTs hold potential utility across various stages of chILD management.
• Well-defined research cohorts are essential to determine the most relevant PFTs and outcomes for chILD.
• Standardized PFT research is crucial for improving clinical care and establishing endpoints for clinical trials in chILD.