Abstract

Childhood Interstitial Lung Disease (chILD) consists of a diverse group of disorders that involve the pulmonary parenchyma and interfere with gas exchange. Patients with chILD may present with respiratory failure, or with more indolent or chronic symptoms including tachypnea, cough, hypoxemia, prolonged respiratory infection, exercise intolerance, or failure to thrive. The differential diagnosis for such a presentation is broad, and it is recommended to exclude more common causes first including infections, immunodeficiency, structural airway abnormalities, congenital heart disease, and cystic fibrosis. Once more common explanations are excluded, a child with unexplained pulmonary symptoms and diffuse pulmonary infiltrates should be given a provisional diagnosis of chILD, and further investigations to determine a specific cause are warranted. In any case, establishing a definitive diagnosis is essential, as it may inform prognosis, genetic counselling for families, and could alter treatment decisions. To date, treatment options for many forms of diffuse lung disease (DLD) are limited and often include drugs of unproven efficacy with substantial side effects. In addition, key differences are present when comparing chILD with adult forms of DLD. Certain diseases are unique to paediatrics and others could present in different proportions with dissimilar prognosis. Thus, chILD presents a diagnostic and therapeutic challenge, even to the most experienced pediatric pulmonologist. In this review we will focus on the presentation, diagnostic approach, treatments, and prognosis of chILD.