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 H M 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":"成年期儿童间质性肺病幸存者：一项欧洲合作研究。","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 H M 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 is rarer in children than adults, but, with increasing diagnostic awareness, more cases are being discovered. The prognosis of childhood interstitial lung disease is often poor, but increasing numbers are now surviving into adulthood.</p><p><strong>Aim: </strong>To characterise childhood interstitial lung disease survivors and identify their impact on adult interstitial lung disease centres.</p><p><strong>Methods: </strong>This was a European study (34 adult and childhood interstitial lung disease centres) reporting incident/prevalent cases of childhood interstitial lung disease survivors from January to July 2023. Epidemiological, clinical, physiological and genetic data were collected.</p><p><strong>Results: </strong>244 patients were identified with a median (interquartile range) age at diagnosis of 12.5 years (6-16 years) and age at study inclusion of 25 years (22-33 years), with 51% male, 86% nonsmokers and a median (interquartile range) % predicted forced vital capacity of 70% (47-89%) and diffusing capacity of the lungs for carbon monoxide of 48% (32-75%). 32% were prescribed long-term oxygen and 227 (93%) were followed up in adult centres whereas 17 (7%) never transitioned. The commonest diagnoses (82%) were childhood interstitial lung disease category B1 (sarcoidosis, hemosiderosis, connective tissue disorders, vasculitis) at 35%, A4 (surfactant-related) at 21%, B2 (bronchiolitis obliterans, hypersensitivity pneumonitis) at 14% and Bz (unclassified interstitial lung disease) at 13%. 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 childhood interstitial lung disease diagnoses were recognised in adult interstitial lung disease classifications.</p><p><strong>Conclusion: </strong>Childhood interstitial lung disease survivors are seen in most adult interstitial lung disease centres and only a minority continue follow-up in paediatric centres. Survivors have a significant loss of lung function. The heterogeneity of their aetiologies and therapeutic requirements has a real impact on adult interstitial lung disease centres. Re-specification of diagnosis and treatment may contribute to precision and personalisation of management.</p>","PeriodicalId":12265,"journal":{"name":"European Respiratory Journal","volume":" ","pages":""},"PeriodicalIF":16.6000,"publicationDate":"2025-02-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"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 H M 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 is rarer in children than adults, but, with increasing diagnostic awareness, more cases are being discovered. The prognosis of childhood interstitial lung disease is often poor, but increasing numbers are now surviving into adulthood.</p><p><strong>Aim: </strong>To characterise childhood interstitial lung disease survivors and identify their impact on adult interstitial lung disease centres.</p><p><strong>Methods: </strong>This was a European study (34 adult and childhood interstitial lung disease centres) reporting incident/prevalent cases of childhood interstitial lung disease survivors from January to July 2023. Epidemiological, clinical, physiological and genetic data were collected.</p><p><strong>Results: </strong>244 patients were identified with a median (interquartile range) age at diagnosis of 12.5 years (6-16 years) and age at study inclusion of 25 years (22-33 years), with 51% male, 86% nonsmokers and a median (interquartile range) % predicted forced vital capacity of 70% (47-89%) and diffusing capacity of the lungs for carbon monoxide of 48% (32-75%). 32% were prescribed long-term oxygen and 227 (93%) were followed up in adult centres whereas 17 (7%) never transitioned. The commonest diagnoses (82%) were childhood interstitial lung disease category B1 (sarcoidosis, hemosiderosis, connective tissue disorders, vasculitis) at 35%, A4 (surfactant-related) at 21%, B2 (bronchiolitis obliterans, hypersensitivity pneumonitis) at 14% and Bz (unclassified interstitial lung disease) at 13%. 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 childhood interstitial lung disease diagnoses were recognised in adult interstitial lung disease classifications.</p><p><strong>Conclusion: </strong>Childhood interstitial lung disease survivors are seen in most adult interstitial lung disease centres and only a minority continue follow-up in paediatric centres. Survivors have a significant loss of lung function. The heterogeneity of their aetiologies and therapeutic requirements has a real impact on adult interstitial lung disease centres. Re-specification of diagnosis and treatment may contribute to precision and personalisation of management.</p>\",\"PeriodicalId\":12265,\"journal\":{\"name\":\"European Respiratory Journal\",\"volume\":\" \",\"pages\":\"\"},\"PeriodicalIF\":16.6000,\"publicationDate\":\"2025-02-13\",\"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\":\"2025/2/1 0:00:00\",\"PubModel\":\"Print\",\"JCR\":\"Q1\",\"JCRName\":\"RESPIRATORY SYSTEM\",\"Score\":null,\"Total\":0}","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":"2025/2/1 0:00:00","PubModel":"Print","JCR":"Q1","JCRName":"RESPIRATORY SYSTEM","Score":null,"Total":0}
Childhood interstitial lung disease survivors in adulthood: a European collaborative study.
Background: Interstitial lung disease is rarer in children than adults, but, with increasing diagnostic awareness, more cases are being discovered. The prognosis of childhood interstitial lung disease is often poor, but increasing numbers are now surviving into adulthood.
Aim: To characterise childhood interstitial lung disease survivors and identify their impact on adult interstitial lung disease centres.
Methods: This was a European study (34 adult and childhood interstitial lung disease centres) reporting incident/prevalent cases of childhood interstitial lung disease survivors from January to July 2023. Epidemiological, clinical, physiological and genetic data were collected.
Results: 244 patients were identified with a median (interquartile range) age at diagnosis of 12.5 years (6-16 years) and age at study inclusion of 25 years (22-33 years), with 51% male, 86% nonsmokers and a median (interquartile range) % predicted forced vital capacity of 70% (47-89%) and diffusing capacity of the lungs for carbon monoxide of 48% (32-75%). 32% were prescribed long-term oxygen and 227 (93%) were followed up in adult centres whereas 17 (7%) never transitioned. The commonest diagnoses (82%) were childhood interstitial lung disease category B1 (sarcoidosis, hemosiderosis, connective tissue disorders, vasculitis) at 35%, A4 (surfactant-related) at 21%, B2 (bronchiolitis obliterans, hypersensitivity pneumonitis) at 14% and Bz (unclassified interstitial lung disease) at 13%. 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 childhood interstitial lung disease diagnoses were recognised in adult interstitial lung disease classifications.
Conclusion: Childhood interstitial lung disease survivors are seen in most adult interstitial lung disease centres and only a minority continue follow-up in paediatric centres. Survivors have a significant loss of lung function. The heterogeneity of their aetiologies and therapeutic requirements has a real impact on adult interstitial lung disease centres. Re-specification of diagnosis and treatment may contribute to precision and personalisation 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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