Understanding Pulmonary Fibrosis in Pediatric Interstitial Lung Disease: A Comprehensive Analysis

Area of Science:

• Pediatric Pulmonology
• Rare Diseases
• Radiology

Background:

• Childhood interstitial lung diseases (chILD) are rare, chronic respiratory conditions.
• Pulmonary fibrosis (PF) is a severe complication associated with mortality in chILD.
• Understanding PF prevalence and characteristics in chILD is critical for patient outcomes.

Purpose of the Study:

• To determine the prevalence and radiologic spectrum of PF in children with chILD.
• To investigate the association between PF and pulmonary function and clinical characteristics.
• To identify risk factors for PF development in chILD patients.

Main Methods:

• A multicenter, retrospective observational cohort study utilizing data from the chILD-TR (as of January 2024).
• Chest CT scans were reviewed by an experienced radiologist for PF findings.
• Patients were grouped based on the presence or absence of PF, with subsequent clinical and demographic data analysis.

Main Results:

• PF findings were present in 183 of 404 chILD patients, with reticular abnormalities being most common (34.9%).
• Patients with PF were older and had lower weight z-scores; DLCO% was significantly lower in the PF group.
• PF was more prevalent in Diffuse Pediatric Lung Disease-A (DPLD-A) disorders compared to DPLD-B.

Conclusions:

• PF is a frequent complication in chILD, with prevalence increasing with age.
• Children with DPLD-A disorders, characterized by surfactant dysfunction or alveolar developmental issues, are at higher risk for PF.
• Lower DLCO, DPLD-A classification, and older age are associated with a higher likelihood of PF in chILD.