Torrent-Vernetta Alba, Gaboli Mirella, Castillo-Corullón Silvia, Mondéjar-López Pedro, Sanz Santiago Verónica, Costa-Colomer Jordi, Osona Borja, Torres-Borrego Javier, de la Serna-Blázquez Olga, Bellón Alonso Sara, Caro Aguilera Pilar, Gimeno-Díaz de Atauri Álvaro, Valenzuela Soria Alfredo, Ayats Roser, Martin de Vicente Carlos, Velasco González Valle, Moure González José Domingo, Canino Calderín Elisa María, Pastor-Vivero María Dolores, Villar Álvarez María Ángeles, Rovira-Amigo Sandra, Iglesias Serrano Ignacio, Díez Izquierdo Ana, de Mir Messa Inés, Gartner Silvia, Navarro Alexandra, Baz-Redón Noelia, Carmona Rosario, Camats-Tarruella Núria, Fernández-Cancio Mónica, Rapp Christina, Dopazo Joaquin, Griese Matthias, Moreno-Galdó Antonio
Paediatric Allergy and Pulmonology Section, Department of Paediatrics, Vall d'Hebron Hospital Universitari, Vall d'Hebron Barcelona Hospital Campus, Universitat Autònoma de Barcelona, Spain; Vall d'Hebron Institut de Recerca (VHIR), Hospital Universitari Vall d'Hebron, Barcelona, Spain; Department of Paediatrics, Obstetrics, Gynecology, Preventative Medicine and Public Health, Universitat Autònoma de Barcelona, Bellaterra, Barcelona, Spain; Center for Biomedical Research on Rare Diseases (CIBERER), Instituto de Salud Carlos III, Spain.
Paediatric Allergy and Pulmonology Section, Department of Paediatrics, University Hospital Virgen del Rocío, Sevilla, Spain.
Arch Bronconeumol. 2022 Jan;58(1):22-29. doi: 10.1016/j.arbres.2021.06.001. Epub 2021 Jun 17.
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. 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.
儿童弥漫性肺疾病，也称为儿童间质性肺疾病（chILD），是一组异质性罕见疾病，具有较高的发病率和死亡率，其诊断和分类非常复杂。流行病学数据匮乏。本研究旨在分析西班牙chILD的发病率和患病率。
对0至18岁患有chILD的患者进行多中心观察性前瞻性研究，基于2018年和2019年报告的数据分析其在西班牙的发病率和患病率。
西班牙各地51个儿科肺病科共报告了381例chILD病例，覆盖了91.7%的儿科人群。chILD的平均发病率为每年每百万儿童8.18例（95%置信区间6.28 - 10.48）。chILD的平均患病率为每百万儿童46.53例（95%置信区间41.81 - 51.62）。患病率最高的年龄组是1岁以下儿童。2至18岁儿童与0至2岁儿童的疾病类型不同。最常见的病例为：新生儿原发性肺间质糖原沉积症（17/65）、1至12个月婴儿的婴儿期神经内分泌细胞增生（44/144）、1至5岁儿童的特发性肺含铁血黄素沉着症（13/74）、5至10岁儿童的过敏性肺炎（9/51）以及10岁以上儿童的硬皮病（8/47）。
我们发现chILD的发病率和患病率高于先前描述的情况，这可能是由于对这些罕见疾病有了更深入的了解以及临床医生的认识提高。
