Approach to the patient with Childhood Interstitial and Diffuse Lung Disease

Abstract

Childhood Interstitial and Diffuse Lung Disease (chILD) encompasses a group of rare, chronic lung disorders in infants and children with overlapping clinical features but diverse etiologies. The clinical presentation of chILD is of chronic or recurring respiratory signs and symptoms, often including increased work of breathing and hypoxia, with diffuse radiographic abnormalities on chest imaging. Recognition can be challenging since some clinical features overlap with those of more common pediatric respiratory diseases including asthma and recurrent viral infections, among others. chILD should be considered as an underlying diagnosis when a patient's respiratory symptoms seem disproportionate to the clinical scenario and/or persist. The diagnostic process involves multiple steps and is tailored to the individual patient. Nearly all children will undergo imaging and pulmonary function testing, many will undergo bronchoscopy with bronchoalveolar lavage, many will receive genetic testing, and some will require lung biopsy. Treatment includes preventive care, evaluation for comorbidities, pharmacotherapy according to diagnosis, and ongoing disease surveillance, including revisiting genetic and histopathologic results as new clinical information becomes available and as our understanding of these rare disorders improves. The purpose of this review is to provide a broad approach to the diagnosis and management of patients with chILD.