The American Thoracic Society (ATS) recently released a 2026 update to its guideline, Classification, Evaluation, and Management of Childhood Interstitial Lung Disease in Infancy. The 2026 guideline update replaces the 2013 version with updated recommendations on lung biopsy, lung transplant referral, chest imaging, and genetic testing. 

Today, we’re highlighting key changes to recommendations made in the 2026 update. View the full-text version of the 2026 ATS childhood interstitial lung diseases (chILD) in infancy guideline for the complete look at the recommendations and their associated backgrounds, evidence bases, evidence levels, and more.

Key Changes in the 2026 Update:
  • Recommendation 1a: Recommend rapid and broad genetic testing for infants and children <2 years with chILD syndrome and respiratory failure for whom potentially fatal chILD disorders are considered (SFTPB, ABCA3, FOXF1). [Strong, very low certainty of evidence]
    • Changes from 2013: Focused on limited number of genes and associated phenotypes with targeted testing based on older methodology. Did not address rapidity of testing.
  • Recommendation 1b: Suggest genetic testing for infants and children <2 years with chILD syndrome be performed prior to invasive diagnostic intervention (e.g. lung biopsy). [Conditional, very low certainty of evidence]
    • Changes from 2013: Genetic testing is recommended under specific circumstances and not as initial approach.
  • Recommendation 1c: Suggest genetic testing for infants and children <2 years with chILD syndrome for whom lung biopsy histopathology features were suggestive of a monogenic chILD disorder. [Conditional, very low certainty of evidence]
    • Changes from 2013: Genetic testing is recommended under specific circumstances and not as initial approach.
  • Recommendation 2: Suggest that CT be part of the diagnostic evaluation for infants and children <2 years with chILD syndrome. [Conditional, very low certainty of evidence]
    • Changes from 2013: Weak recommendation to characterize nature and distribution of lung disease.
  • Recommendation 3a: Suggest surgical lung biopsy for infants and children <2 years with chILD syndrome for whom other diagnostic interventions have not identified a specific chILD disorder and whose critical condition is not improving. [Conditional, very low certainty of evidence]
    • Changes from 2013: Strong recommendation for VATS over open approach if expertise is available. Unspecified recommendation to follow published protocols for handling and analysis of specimen.
  • Recommendation 3b: Recommend surgical lung biopsy for infants and children <2 years with chILD syndrome for whom there is clinical urgency to identify a specific chILD disorder and genetic testing is not feasible, inconclusive or timely, to identify histopathological “treatable traits”. [Strong, very low certainty of evidence]
    • Changes from 2013: Strong recommendation for biopsy if other diagnostic studies have not identified cause or clinical urgency. Identification of “treatable traits” not addressed.
  • Recommendation 4a: Recommend early referral for lung transplant evaluation in infants and children with specific monogenic chILD disorders with predictable poor outcomes. [Strong, very low certainty of evidence]
    • Changes from 2013: Strong recommendation for referral based on “life-threatening” chILD diseases.
  • Recommendation 4b: Suggest early referral for lung transplant evaluation in infants for infants and children with chILD syndrome requiring mechanical ventilation or ECMO or with progressive pulmonary hypertension, or a specific chILD disorder with progressive respiratory symptoms or impending respiratory failure. [Conditional, very low certainty of evidence]
    • Changes from 2013: Strong recommendation for referral based on “life-threatening” chILD diseases.
  • Recommendation 5: Suggest repeat chest CT to aid decision making, prognosis, or change therapies targeting specific “treatable traits” (e.g. fibrosis, inflammation). [Conditional, very low certainty of evidence]
    • Changes from 2013: Not addressed.

The ATS noted that the 2013 guideline used different methodology, which affected the strength of recommendations. 

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