Effrosyni D Manali, Matthias Griese, Nadia Nathan, Yurdagül Uzunhan, Raphael Borie, Katarzyna Michel, Nicolaus Schwerk, Justyna Fijolek, Elżbieta Radzikowska, Felix Chua, Rishi Pabary, Nesrin Mogulkoc, Cormac McCarthy, Maria Kallieri, Andriana I Papaioannou, Nural Kiper, Martina Koziar Vasakova, Ladislav Lacina, Maria Molina-Molina, Alba Torrent-Vernetta, Theofanis Tsiligiannis, Bulent Karadag, Maria Kokosi, Elisabetta A Renzoni, Coline Hm van Moorsel, Ilaria Campo, Elisabeth Bendstrup, Thomas Skovhus Prior, Antje Prasse, Francesco Bonella, Vincent Cottin, Rémi Diesler, Antoine Froidure, Lykourgos Kolilekas, Lampros Fotis, Konstantinos Douros, Athanasios G Kaditis, Florence Jeny, Simon Chauveau, Hilario Nunes, Azrine Dahbia, Francesca Mariani, Joanne J van der Vis, Karlijn Groen, Ela Erdem Eralp, Yasemin Gokdemir, Derya Kocakaya, Sehnaz Olgun Yildizeli, Ebru Yalçın, Nagehan Emiralioğlu, Halime Nayir Buyuksahin, Helen O'Brien, Oguz Karcıoglu, Demet Can, Alper Ezircan, Gokcen Kartal Ozturk, Nesrin Ocal, Hasan Yuksel, Sedef Narin Tongal, Martina Safrankova, Katerina Kourtesi, Camille Louvrier, Caroline Kannengiesser, Aurelie Fabre, Marie Legendre, Bruno Crestani, Petr Pohunek, Andrew Bush, Spyros A Papiris
{"title":"Childhood interstitial lung disease survivors in adulthood: a European collaborative study.","authors":"Effrosyni D Manali, Matthias Griese, Nadia Nathan, Yurdagül Uzunhan, Raphael Borie, Katarzyna Michel, Nicolaus Schwerk, Justyna Fijolek, Elżbieta Radzikowska, Felix Chua, Rishi Pabary, Nesrin Mogulkoc, Cormac McCarthy, Maria Kallieri, Andriana I Papaioannou, Nural Kiper, Martina Koziar Vasakova, Ladislav Lacina, Maria Molina-Molina, Alba Torrent-Vernetta, Theofanis Tsiligiannis, Bulent Karadag, Maria Kokosi, Elisabetta A Renzoni, Coline Hm van Moorsel, Ilaria Campo, Elisabeth Bendstrup, Thomas Skovhus Prior, Antje Prasse, Francesco Bonella, Vincent Cottin, Rémi Diesler, Antoine Froidure, Lykourgos Kolilekas, Lampros Fotis, Konstantinos Douros, Athanasios G Kaditis, Florence Jeny, Simon Chauveau, Hilario Nunes, Azrine Dahbia, Francesca Mariani, Joanne J van der Vis, Karlijn Groen, Ela Erdem Eralp, Yasemin Gokdemir, Derya Kocakaya, Sehnaz Olgun Yildizeli, Ebru Yalçın, Nagehan Emiralioğlu, Halime Nayir Buyuksahin, Helen O'Brien, Oguz Karcıoglu, Demet Can, Alper Ezircan, Gokcen Kartal Ozturk, Nesrin Ocal, Hasan Yuksel, Sedef Narin Tongal, Martina Safrankova, Katerina Kourtesi, Camille Louvrier, Caroline Kannengiesser, Aurelie Fabre, Marie Legendre, Bruno Crestani, Petr Pohunek, Andrew Bush, Spyros A Papiris","doi":"10.1183/13993003.00680-2024","DOIUrl":null,"url":null,"abstract":"<p><strong>Background: </strong>Interstitial lung disease (ILD) is rarer in children (chILD) than adults, but with increasing diagnostic awareness, more cases are being discovered. chILD prognosis is often poor, but increasing numbers are now surviving into adulthood.</p><p><strong>Aim: </strong>To characterize chILD-survivors and identify their impact on adult-ILD centers.</p><p><strong>Methods: </strong>European study (34 adult-ILD and chILD centers) reporting incident/prevalent cases of chILD-survivors from January to July 2023. Epidemiological, clinical, physiological and genetic data were collected.</p><p><strong>Results: </strong>244 patients were identified with median (years) (IQR) age at diagnosis 12.5 (6-16), age at study inclusion 25 (22-33), 51% male, 86% non-smokers, median %-predicted FVC and DLCO 70 (47-89) and 48 (32-75) respectively; 32% were prescribed long-term oxygen; 227 (93%) were followed-up in adult centers whereas 17 (7%) never transitioned. Commonest diagnoses (82%) were chILD category B1, 35% (sarcoidosis, hemosiderosis, connective tissue disorders, vasculitis); A4, 21% (surfactant-related); B2, 14% (bronchiolitis obliterans, hypersensitivity pneumonitis); Bz, 13% (unclassified-ILD). Bz patients had the worst functional status. 60% of all patients were still being prescribed corticosteroids. Re-specification of diagnosis and treatment were made after transition for 9.8% and 16% of patients respectively. Not all chILD diagnoses were recognized in adult-ILD classifications.</p><p><strong>Conclusion: </strong>chILD survivors are seen in most adult-ILD centers and only a minority continue follow-up in pediatric centers. Survivors have a significant loss of lung function. The heterogeneity of their etiologies and therapeutic requirements has a real impact on adult-ILD centers. Re-specification of diagnosis and treatment may contribute to precision and personalization of management.</p>","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":16.6000,"publicationDate":"2024-12-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"European Respiratory Journal","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1183/13993003.00680-2024","RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"RESPIRATORY SYSTEM","Score":null,"Total":0}
引用次数: 0
Abstract
Background: Interstitial lung disease (ILD) is rarer in children (chILD) than adults, but with increasing diagnostic awareness, more cases are being discovered. chILD prognosis is often poor, but increasing numbers are now surviving into adulthood.
Aim: To characterize chILD-survivors and identify their impact on adult-ILD centers.
Methods: European study (34 adult-ILD and chILD centers) reporting incident/prevalent cases of chILD-survivors from January to July 2023. Epidemiological, clinical, physiological and genetic data were collected.
Results: 244 patients were identified with median (years) (IQR) age at diagnosis 12.5 (6-16), age at study inclusion 25 (22-33), 51% male, 86% non-smokers, median %-predicted FVC and DLCO 70 (47-89) and 48 (32-75) respectively; 32% were prescribed long-term oxygen; 227 (93%) were followed-up in adult centers whereas 17 (7%) never transitioned. Commonest diagnoses (82%) were chILD category B1, 35% (sarcoidosis, hemosiderosis, connective tissue disorders, vasculitis); A4, 21% (surfactant-related); B2, 14% (bronchiolitis obliterans, hypersensitivity pneumonitis); Bz, 13% (unclassified-ILD). Bz patients had the worst functional status. 60% of all patients were still being prescribed corticosteroids. Re-specification of diagnosis and treatment were made after transition for 9.8% and 16% of patients respectively. Not all chILD diagnoses were recognized in adult-ILD classifications.
Conclusion: chILD survivors are seen in most adult-ILD centers and only a minority continue follow-up in pediatric centers. Survivors have a significant loss of lung function. The heterogeneity of their etiologies and therapeutic requirements has a real impact on adult-ILD centers. Re-specification of diagnosis and treatment may contribute to precision and personalization of management.
期刊介绍：
The European Respiratory Journal (ERJ) is the flagship journal of the European Respiratory Society. It has a current impact factor of 24.9. The journal covers various aspects of adult and paediatric respiratory medicine, including cell biology, epidemiology, immunology, oncology, pathophysiology, imaging, occupational medicine, intensive care, sleep medicine, and thoracic surgery. In addition to original research material, the ERJ publishes editorial commentaries, reviews, short research letters, and correspondence to the editor. The articles are published continuously and collected into 12 monthly issues in two volumes per year.
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