PT - JOURNAL ARTICLE AU - Alba Torrent Vernetta AU - Sandra Rovira-Amigo AU - Ignacio Iglesias Serrano AU - Ana Díez Isquierdo AU - Matthias Griese AU - Christina Rapp AU - Mirella Gaboli AU - Silvia Castillo-Corullon AU - Pedro Mondejar-Lopez AU - Verónica Sanz Santiago AU - Jordi Costa-Colomer AU - Borja Osona AU - Javier Torres-Borrego AU - Olga De La Serna Blázquez AU - Sara Bellon Alonso AU - Pilar Caro Aguilera AU - Álvaro Gimeno-Diaz De Atauri AU - Alfredo Valenzuela Soria AU - Roser Ayats AU - Carlos Martin De Vicente AU - Valle Velasco Gonzalez AU - Jose Domingo Moure Gonzalez AU - Antonio Moreno-Galdo TI - Incidence and prevalence of children’s diffuse lung disease in Spain AID - 10.1183/13993003.congress-2021.OA4073 DP - 2021 Sep 05 TA - European Respiratory Journal PG - OA4073 VI - 58 IP - suppl 65 4099 - http://erj.ersjournals.com/content/58/suppl_65/OA4073.short 4100 - http://erj.ersjournals.com/content/58/suppl_65/OA4073.full SO - Eur Respir J2021 Sep 05; 58 AB - Background: Children’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, and epidemiological data are scarce.Objective: To analyse incidence and prevalence of chILD in Spain.Methods: Multicentre observational prospective study in patients under 18 years of age with chILD during 2018 and 2019 in Spain.Results: A total of 380 cases with chILD were notified from 51 paediatric pulmonology units all over the country, covering a paediatric population of 7,644,155 in 2018 and 7,636,093 in 2019. The average incidence of paediatric diffuse lung disease was 8.18 new cases/million of children per year (CI95% 6.28-10.48). The average prevalence of paediatric diffuse lung disease was 46.39 cases/million of children (CI95% 41.69-51.48). The age group with the highest prevalence were children under 1 year of age. Different types of disorders were seen in children 2 to 18 years of age compared with children 0 to 2 years of age. Most frequent cases were: primary pulmonary interstitial glycogenosis in neonates (14/62), neuroendocrine cell hyperplasia of infancy in infants (44/144), idiopathic pulmonary hemosiderosis in children 1-5 years old (13/74), hypersensitivity pneumonia in children 5-10 years old (9/52), and scleroderma in older than 10 years old (8/48). The 9.5% (36/380) of cases had a disease causing mutation, and same number (36/380) had no clear aetiology.Conclusions: We found an incidence and prevalence of chILD much higher than previously described probably due to greater understanding and increased clinician awareness of these rare diseases.FootnotesCite this article as: European Respiratory Journal 2021; 58: Suppl. 65, OA4073.This abstract was presented at the 2021 ERS International Congress, in session “Prediction of exacerbations in patients with COPD”.This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).