Torrent-Vernetta, AlbaGaboli, MirellaCastillo-Corullón, SilviaMondéjar-López, PedroSanz Santiago, VerónicaCosta-Colomer, JordiOsona, BorjaTorres-Borrego, Javierde la Serna-Blázquez, OlgaBellón Alonso, SaraCaro Aguilera, PilarGimeno-Díaz de Atauri, ÁlvaroValenzuela Soria, AlfredoAyats, RoserMartin de Vicente, CarlosVelasco González, ValleMoure González, José DomingoCanino Calderín, Elisa MaríaPastor-Vivero, María DoloresVillar Álvarez, María ÁngelesRovira-Amigo, SandraIglesias Serrano, IgnacioDíez Izquierdo, Anade Mir Messa, InésGartner, SilviaNavarro, AlexandraBaz-Redón, NoeliaCarmona, RosarioCamats-Tarruella, NúriaFernández-Cancio, MónicaRapp, ChristinaDopazo, JoaquinGriese, MatthiasMoreno-Galdó, AntonioChILD-Spain Group2023-05-032023-05-032021-06-17http://hdl.handle.net/10668/21993Children's diffuse lung disease, also known as children's Interstitial Lung Diseases (chILD), are a heterogeneous group of rare diseases with relevant morbidity and mortality, which diagnosis and classification are very complex. Epidemiological data are scarce. The aim of this study was to analyse incidence and prevalence of chILD in Spain. Multicentre observational prospective study in patients from 0 to 18 years of age with chILD to analyse its incidence and prevalence in Spain, based on data reported in 2018 and 2019. A total of 381 cases with chILD were notified from 51 paediatric pulmonology units all over Spain, covering the 91.7% of the paediatric population. The average incidence of chILD was 8.18 (CI 95% 6.28-10.48) new cases/million of children per year. The average prevalence of chILD was 46.53 (CI 95% 41.81-51.62) cases/million of children. The age group with the highest prevalence were children under 1 year of age. Different types of disorders were seen in children 2-18 years of age compared with children 0-2 years of age. Most frequent cases were: primary pulmonary interstitial glycogenosis in neonates (17/65), neuroendocrine cell hyperplasia of infancy in infants from 1 to 12 months (44/144), idiopathic pulmonary haemosiderosis in children from 1 to 5 years old (13/74), hypersensitivity pneumonitis in children from 5 to 10 years old (9/51), and scleroderma in older than 10 years old (8/47). We found a higher incidence and prevalence of chILD than previously described probably due to greater understanding and increased clinician awareness of these rare diseases.enChildhood interstitial lung diseaseChildrenChildren's Interstitial Lung diseaseDiffuse lung diseaseEnfermedad pulmonar intersticial infantilEnfermedades difusas del parénquima pulmonar en pediatríaNeumopatías intersticiales en pediatríaNiñosPaediatricsPediatríachILDresearch article35249699open access10.1016/j.arbres.2021.06.0011579-2129https://doi.org/10.1016/j.arbres.2021.06.001