Children interstitial lung disease: Assessment and management.

Diffuse parenchymal lung diseases or children interstitial lung disease (chILD) in pediatrics are a heterogenous group of more than 200 rare diseases with an incidence and prevalence around 8.2 and 46.4 cases/million, respectively, higher than previously recognized, probably related to a greater understanding and increased clinical awareness of these diseases. Children under 2 years of age account for 40%-60% of cases. Diseases presenting before the age of 2 years are mainly of genetic origin or associated with disorders of lung development and are very different from those of older children and adults. In 10%-20% of cases, a genetic cause is found, and in approximately 12%, no cause (undefined chILD) is found after performing all diagnostic tests. A multidisciplinary approach in an expert center is essential for diagnosis and treatment. Tests to be performed after lung computed tomography include serological and other blood tests, bronchoscopy and bronchoalveolar lavage, genetic studies and lung biopsy. Although no approved specific treatment for most cases of chILD exists, an improved understanding of the pathophysiology of many of these diseases is facilitating the development of new treatments.