ATS publishes clinical practice guideline on the classification, evaluation, and management of childhood interstitial lung disease in infancy
The American Thoracic Society has released new clinical practice guidelines on the classification, evaluation and management of childhood interstitial lung disease (chILD) in infants. Childhood ILD includes a diverse group of rare lung diseases found in infants, children and teens that involve the interstitial tissues of the lung, which surround the air sacs (alveoli) in the lung and airways (breathing tubes). It is not known how many children have these disorders. Some types of chILD are caused by other diseases, while the cause is unclear in others, and prognosis varies by disease type.
The new guidelines appear in the August 1 American Journal of Respiratory and Critical Care Medicine.
“The characteristics and natural history of ILD differ in children and adults,” said Geoffrey Kurland, MD, of the Division of Pediatric Pulmonology at the Children’s Hospital of Pittsburgh and a member of the committee that drafted the guidelines. “These new guidelines specifically address the diagnostic evaluation and management of chILD in neonates and infants, as most of the new diagnostic entities that have been recently described affect infants disproportionately.”
The guidelines’ recommendations include the following:
- Once other possible causes of lung disease symptoms have been eliminated, “chILD Syndrome” can be diagnosed if at least three of the following four criteria are present: (1) respiratory symptoms (cough, rapid and/or difficult breathing, or exercise intolerance), (2) respiratory signs (tachypnea, adventitious sounds, retractions, digital clubbing, failure to thrive, or respiratory failure), (3) hypoxemia, and (4) diffuse abnormalities on a chest radiograph or computed tomography (CT) scan.
- In patients with chILD Syndrome, diagnostic testing should be performed to determine the exact chILD diagnosis and echocardiography should be performed as part of the initial evaluation to rule out structural cardiovascular disease and pulmonary hypertension. If thin section CT is performed, the lowest radiation dose that provides adequate diagnostic information should be used.
- For neonates and infants with chILD Syndrome in whom other diagnostic investigations have not identified the precise chILD disease, or in whom there is clinical urgency to identify the precise chILD disease, surgical lung biopsy should be performed, preferably using video-assisted thoracoscopy rather than open thoracotomy.
- Appropriate genetic testing should be performed.
An Official ATS Clinical Practice Guideline: Classification, Evaluation, and Management of Childhood Interstitial Lung Disease (chILD) in Infancy (pdf), Geoffrey Kurland, Robin R Deterding, James S Hagood, Lisa RYoung, Alan S Brody, Robert G Castile, Sharon Dell, Leland L Fan, Aaron Hamvas, Bettina C Hilman, Claire Langston, Lawrence M Nogee, Gregory J Redding
American Thoracic Society