Interstitial lung disease in children.

Area of Science:

• Pediatric Pulmonology
• Rare Diseases
• Interstitial Lung Diseases

Background:

• Interstitial lung diseases (ILDs) in children are rare, diffuse lung diseases with diverse etiologies.
• Causes include genetic factors, systemic diseases, and inflammatory or fibrotic responses.
• Unique presentations and causes are observed in infancy.

Purpose of the Study:

• To review recent advances in understanding pediatric interstitial lung disease (ILD).
• To cover the cause, pathogenesis, presentation, diagnosis, treatment, and prognosis of childhood ILD.

Main Methods:

• Review of current literature on pediatric ILD.
• Discussion of classification schemes for ILD in children.
• Evaluation of diagnostic approaches, including noninvasive and invasive studies.

Main Results:

• ILD classification in children >2 years often uses adult schemes; pathological evaluation is key for <2 years.
• Pathological categories include developmental, growth, and surfactant dysfunction disorders.
• Neuroendocrine cell hyperplasia of infancy and pulmonary interstitial glycogenosis have favorable prognoses.

Conclusions:

• Pediatric ILDs stem from various pathogenic processes, with distinct infantile forms.
• Diagnosis integrates clinical, radiologic, and pathological findings.
• Ongoing research aims to minimize the necessity of lung biopsies.