Interstitial lung diseases (ILDs) in children constitute a heterogeneous group of rare diseases that have been described and classified according to experiences and research in adults. However, pediatric pulmonologists have observed that the clinical spectrum is broader in children than in adults, and that many of these disorders have different courses and treatment responses. In addition, probably due to the various stages of lung development and maturation, new clinical forms have been described, particularly in infants. This has broadened the classification of ILDs in this age bracket. The understanding that neither the usual definition nor the standard classification of these disorders entirely apply to children has prompted multicenter studies designed to increase knowledge of these disorders, as well as to standardize diagnostic and therapeutic strategies. We have reviewed the conceptualization of ILDs in children, taking into consideration the particularities of this group of patients when using the criteria for the classification of these diseases in adults. We have also made a historical review of several multicenter studies in order to further understanding of the problem. We have emphasized the differences in the clinical presentation, in an attempt to highlight knowledge of newly described entities in young children. We underscore the need to standardize management of laboratory and radiological routines, as well as of lung biopsy processing, taking such knowledge into account. It is important to bear in mind that, among the recently described disorders, genetic surfactant dysfunction, which is often classified as an idiopathic disease in adults, should be included in the differential diagnosis of ILDs.

Keywords: Lung diseases, interstitial; Lung diseases, interstitial/diagnosis; Lung diseases, interstitial/therapy; Child.