Childhood interstitial lung disease survivors in adulthood: a European collaborative study.
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Manali Effrosyni D, Griese Matthias, Nathan Nadia, Uzunhan Yurdagül, Borie Raphael, Michel Katarzyna, Schwerk Nicolaus, Fijolek Justyna, Radzikowska Elżbieta, Chua Felix, Pabary Rishi, Mogulkoc Nesrin, McCarthy Cormac, Kallieri Maria, Papaioannou Andriana I, Kiper Nural, Koziar Vasakova Martina, Lacina Ladislav, Molina-Molina Maria, Torrent-Vernetta Alba, Tsiligiannis Theofanis, Karadag Bulent, Kokosi Maria, Renzoni Elisabetta A, van Moorsel Coline H M, Campo Ilaria, Bendstrup Elisabeth, Prior Thomas Skovhus, Prasse Antje, Bonella Francesco, Cottin Vincent, Diesler Rémi, Froidure Antoine, Kolilekas Lykourgos, Fotis Lampros, Douros Konstantinos, Kaditis Athanasios G, Jeny Florence, Chauveau Simon, Nunes Hilario, Dahbia Azrine, Mariani Francesca, van der Vis Joanne J, Groen Karlijn, Erdem Eralp Ela, Gokdemir Yasemin, Kocakaya Derya, Olgun Yildizeli Sehnaz, Yalçın Ebru, Emiralioğlu Nagehan, Nayir Buyuksahin Halime, O'Brien Helen, Karcıoglu Oguz, Can Demet, Ezircan Alper, Kartal Ozturk Gokcen, Ocal Nesrin, Yuksel Hasan, Narin Tongal Sedef, Safrankova Martina, Kourtesi Katerina, Louvrier Camille, Kannengiesser Caroline, Fabre Aurelie, Legendre Marie, Crestani Bruno, Pohunek Petr, Bush Andrew, Papiris Spyros A
Аннотация
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.