Application of clinico-radiologic-pathologic diagnosis of diffuse parenchymal lung diseases in children in China

Area of Science:

• Pediatric Pulmonology
• Respiratory Medicine
• Pediatric Pathology

Background:

• Diffuse parenchymal lung diseases in children (chDPLD), also known as interstitial lung diseases in children (chILD), are chronic respiratory disorders with significant morbidity and mortality.
• The low incidence of chDPLD results in limited pediatrician knowledge, particularly in China.
• Accurate diagnosis and characterization of chDPLD are essential for effective management.

Purpose of the Study:

• To describe the clinico-radiologic-pathologic (CRP) characteristics of chDPLD in China.
• To determine the prevalence of different subtypes of chDPLD within the Chinese pediatric population.
• To enhance understanding of this rare disease group in a specific geographic context.

Main Methods:

• A retrospective analysis of 25 pediatric cases diagnosed with chDPLD across 16 hospitals in China.
• Data collection included clinical presentations, radiographic findings, and histopathologic results from lung biopsies.
• Cases were categorized based on etiology, including exposure-related, systemic disease-associated, and alveolar structure disorder-associated ILD.

Main Results:

• The study identified 25 cases of chDPLD (18 boys, 7 girls) with a median age of 6.0 years.
• Common symptoms included cough (96%), dyspnea (84%), and fever (16%).
• Non-specific interstitial pneumonia was the most frequent diagnosis (n=9), followed by alveolar structure disorder-associated ILD (n=19).

Conclusions:

• Non-specific interstitial pneumonia represents the predominant category of chDPLD in China.
• Lung biopsy remains a critical diagnostic tool for definitive chDPLD classification.
• Careful evaluation of clinical and imaging data is necessary to support specific chDPLD diagnoses.