The American Journal of Respiratory and Critical Care Medicine (AJRCCM)An Update to the Classification, Evaluation, and Management of Childhood Interstitial Lung Disease in Infancy: An Official American Thoracic Society Clinical Practice Guideline

✅ Guideline Update

The ATS guideline updates classification, evaluation, and management recommendations for children younger than 2 years with childhood interstitial lung disease (chILD). The document addresses genetic testing, chest CT, lung biopsy, repeat imaging, and transplant referral.

Clinical Considerations

• Rapid, broad genetic testing is strongly recommended for infants with chILD and respiratory failure.
• Chest CT remains important, while repeat CT may support prognosis, therapy decisions, or disease monitoring.
• Surgical lung biopsy is strongly recommended when genetic testing is infeasible, delayed, or inconclusive.
• Transplant evaluation is strongly recommended for chILD disorders with predictably poor outcomes.

Practice Applications

• Integrate updated ATS guidance into infant chILD diagnostic pathways.
• Recognize genetic testing as a prioritized evaluation step.
• Interpret biopsy use through urgency, feasibility, and prognosis.
• Monitor referral timing for transplant-eligible high-risk disorders.