An Update to the Classification, Evaluation, and Management of Childhood Interstitial Lung Disease in Infancy: An Official American Thoracic Society Clinical Practice Guideline

BACKGROUND: Childhood interstitial lung diseases (chILD) are a heterogeneous group of rare disorders. In 2013, the American Thoracic Society (ATS) developed a guideline regarding the classification, evaluation, and management of children <2 years old with chILD. The current guideline provides updated recommendations regarding genetic testing, chest imaging, lung biopsy, and lung transplant referral. METHODS: The Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) approach was used to form clinical questions, summarize evidence, and develop recommendations, following ATS policies and procedures. A multidisciplinary panel with expertise in chILD formulated recommendations addressing diagnostic tools and referral for lung transplant evaluation. RESULTS: Rapid and broad genetic testing is recommended for infants with chILD and respiratory failure (strong recommendation). Chest CT remains an important part of the diagnostic evaluation (conditional recommendation). Surgical lung biopsy is recommended when there is urgency to identify a specific chILD disorder and genetic testing is not feasible, timely, or inconclusive, to identify histopathologic 'treatable traits', or to inform prognosis (strong recommendation). Referral for lung transplant evaluation is recommended for children with specific chILD disorders with predictably poor outcomes (strong recommendation). Repeat chest CT to aid decision-making, prognosis, and/or change therapies is suggested for children with chILD diagnosed before age 2 years (conditional recommendation). CONCLUSIONS: This guideline provides evidence-based recommendations for genetic testing, chest imaging, lung biopsy, and referral for lung transplant evaluation to improve the diagnostic evaluation and management of children <2 years with chILD. Implementing this guideline will hopefully increase genetic testing for chILD, inform the diagnostic evaluation and disease monitoring of chILD, and identify future research priorities for chILD.

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Publication Details

Journal
American Journal of Respiratory and Critical Care Medicine
Published
2026-07-24
DOI
https://doi.org/10.1093/ajrccm/aamag375
Primary Topic
Neonatal Respiratory Health Research
Type
article
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article

An Update to the Classification, Evaluation, and Management of Childhood Interstitial Lung Disease in Infancy: An Official American Thoracic Society Clinical Practice Guideline

Gloria Pryhuber, Duc Ha, Lisa R. Young, Joshua Blatter et al.
American Journal of Respiratory and Critical Care Medicine
Neonatal Respiratory Health Research
article

An Update to the Classification, Evaluation, and Management of Childhood Interstitial Lung Disease in Infancy: An Official American Thoracic Society Clinical Practice Guideline

Gloria Pryhuber, Duc Ha, Lisa R. Young, Joshua Blatter, Emily M. DeBoer, Robin R. Deterding, Martha P. Fishman, William A. Gower, Sara O. Vargas, Sharon Dell, Narayan P. Iyer, Vivek Balasubramaniam, Gail H. Deutsch, James S. Hagood, Lawrence M. Nogee, Velda Ocasio Ramírez, G. Pryhuber, R. Epaud, Hayley Barnes, Jennifer Pogoriler, Maureen B. Josephson, Elizabeth K. Fiorino, Daniel Craven, Prangthip Charoenpong, Steven K. Brennan, Samuel Goldfarb, Timothy J. Vece, Aaron Hamvas, Jason P. Weinman, S. Christy Sadreameli, Nadia Nathan, Rebekah J. Nevel, Jennifer Wambach, Benjamin A. Raby, Adam Jaffé, Matthew McGraw, Alicia Casey, John C. Kennedy, Deborah R. Liptzin, S Dell, Alejandro Teper, Ralph Epaud, Ann Gettys, Katelyn Krivchenia, Hayley Barnes, A Hamvas, Adam Jaffe, Geoffrey Kurland, Jennifer Pogoriler, A M Casey, A M Teper, L M Nogee, J A Wambach, S B Goldfarb, L R Young, Maureen B. Parenti, Emily DeBoer, Daniel Craven, the American Thoracic Society Assembly on Pediatrics, Velda Ocasio-Ramirez, Nadia Nathan, Joshua Blatter
article en

Abstract

BACKGROUND: Childhood interstitial lung diseases (chILD) are a heterogeneous group of rare disorders. In 2013, the American Thoracic Society (ATS) developed a guideline regarding the classification, evaluation, and management of children <2 years old with chILD. The current guideline provides updated recommendations regarding genetic testing, chest imaging, lung biopsy, and lung transplant referral. METHODS: The Grading of Recommendations, Assessment, Development, and Evaluation (GRADE) approach was used to form clinical questions, summarize evidence, and develop recommendations, following ATS policies and procedures. A multidisciplinary panel with expertise in chILD formulated recommendations addressing diagnostic tools and referral for lung transplant evaluation. RESULTS: Rapid and broad genetic testing is recommended for infants with chILD and respiratory failure (strong recommendation). Chest CT remains an important part of the diagnostic evaluation (conditional recommendation). Surgical lung biopsy is recommended when there is urgency to identify a specific chILD disorder and genetic testing is not feasible, timely, or inconclusive, to identify histopathologic 'treatable traits', or to inform prognosis (strong recommendation). Referral for lung transplant evaluation is recommended for children with specific chILD disorders with predictably poor outcomes (strong recommendation). Repeat chest CT to aid decision-making, prognosis, and/or change therapies is suggested for children with chILD diagnosed before age 2 years (conditional recommendation). CONCLUSIONS: This guideline provides evidence-based recommendations for genetic testing, chest imaging, lung biopsy, and referral for lung transplant evaluation to improve the diagnostic evaluation and management of children <2 years with chILD. Implementing this guideline will hopefully increase genetic testing for chILD, inform the diagnostic evaluation and disease monitoring of chILD, and identify future research priorities for chILD.

American Journal of Respiratory and Critical Care Medicine
University of North Carolina at Chapel Hill (US), Boston Children's Hospital (US), The University of Sydney (AU), University of Minnesota (US), University of Wisconsin System (US), Nationwide Children's Hospital (US), St. Louis Children's Hospital (US), Children's Hospital of Philadelphia (US), Children's Hospital of Los Angeles (US), University of Wisconsin–Madison (US), University of British Columbia (CA), Johns Hopkins University (US), Inserm (FR), University of California, San Francisco (US), Baylor College of Medicine (US), Université Paris-Est Créteil (FR), University of Washington (US), Johns Hopkins Medicine (US), University of Missouri Hospital (US), University of Rochester Medical Center (US), Sorbonne Université (FR), Northwestern Memorial Hospital (US), Donald & Barbara Zucker School of Medicine at Hofstra/Northwell (US), Seattle Children's Hospital (US), Laboratoire National de Référence (MA), Lurie Children's Hospital (US), Children's Hospital of Pittsburgh (US), Pulmonary Associates (US), Boston Children's Museum (US), Rainbow Babies & Children's Hospital (US), Hôpital Intercommunal de Créteil (FR), Hospital General de Niños Ricardo Gutierrez (AR), Robert H. Lurie Comprehensive Cancer Center of Northwestern University, University of Colorado Anschutz Medical Campus (US), University of San Francisco (US), University of Oklahoma (US), University of Colorado Denver (US)
Openalex Percentile: Top 9%
Neonatal Respiratory Health Research
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