Guidelines for infant interstitial lung disease

The American Thoracic Society has published new guidelines on the classification, diagnosis, and management of childhood interstitial lung disease (chILD), focusing on neonates and infants aged younger than 2 years.

The guidelines are important because the causes of ILD in infants are often different from those in older children and adults. As a result, the course and outcomes also vary, so getting a correct diagnosis is vital for effective treatment.

In writing the guidelines, the multidisciplinary panel focused on the accuracy of the diagnostic evaluation, the consequences of a delayed or incorrect diagnosis, and the psychosocial components affecting the patient’s (and family’s) quality of life and, possibly, death.

Specifically, the guidelines review the clinical characteristics of babies aged younger than 2 years with diffuse lung disease (DLD); the common causes of DLD that should be eliminated; methods for further clinical investigation of infants who are lumped into the “childhood ILD syndrome” category; a new pathologic classification scheme of DLD in infants; supportive and continuing care; and areas for future research.

According the National Heart, Lung, and Blood Institute, experts still do not know how many children have chILD, partly because diagnosis is difficult and partly because it is a broad term for a group of diseases with similar symptoms and not a precise diagnosis.

Some forms in children are the same as those in adults, such as hypersensitivity pneumonitis, immunodeficiency-associated lung disease, and bronchiolitis obliterans, but other forms occur only in children, including lung growth abnormalities, neuroendocrine cell hyperplasia of infancy (NEHI), pulmonary interstitial glycogenosis, and developmental disorders, such as alveolar capillary dysplasia.

Because each form of chILD may differ in its severity and treatment, the authors of the guidelines conclude that neonates and infants with childhood ILD syndrome be evaluated by a knowledgeable subspecialist.

For more information for parents of children with ILD, pediatricians can download resources from the American Thoracic Society’s patient information series on chILD.

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