Analysis of children’s diffuse parenchymal lung disease from the European Management Platform for Childhood Interstitial Lung Diseases: Frequency of disease categories and treatments used

Introduction: Children’s interstitial lung disease (chILD) comprises a heterogeneous group of over 200 different entities of diffuse lung diseases with symptom onset in childhood. Only 1-3 of one million children are affected per year. No evidence based treatment guidelines exist. Aim: First analysis of the distribution of chILD disease entities using a classification system also including cases without lung biopsy (Orphanet J Rare Dis 2015 10:122), as well as respiratory medications prescribed. Methods: Patients with chILD reported to the European Management Platform for Childhood Interstitial Lung Diseases between December 2012 and November 2016 were included. Baseline and follow up visits including information on disease course and respiratory treatments used in the whole cohort and specific subcategories were analysed. Results: 366 peer-reviewed patients received a final diagnosis: the most common was chILD related to alveolar surfactant region (category A4, n=194), followed by chILD related to systemic disease (B1, n=60), specific conditions of undefined etiology (A3, n=59) and chILD of the immune intact host (B2, n=46). The most commonly used drugs at baseline were glucocorticosteroids in 175 cases (48%), especially in the subcategories A4 and B1. Azithromycin was used in 24% and hydroxychloroquine in 14%. Conclusion: The knowledge of most frequently diagnosed subcategories of chILD and most commonly used medication in these groups will inform controlled studies in the future, especially for glucocorticosteroids and azithromycin in the subcategories A4 and B1.