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

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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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