Children’s ILDs survivors in adulthood: a European Study

<bold>Background:</bold> Children’s ILDs (chILD) often have a bad prognosis, but many survive into adulthood. <bold>Aim:</bold> To characterize chILD-survivors and identify their burden in adult-ILD centers. <bold>Methods:</bold> European study (33 adult-ILD and chILD centers) reporting incident/prevalent cases of chILD-survivors, January-July 2023. Epidemiological, clinical, physiologic, genetic data were collected. <bold>Results:</bold> Identified were 247 patients, median age at inclusion (IQR 25-75; years) of 25 (22-34), 50.2% male, 86% non-smokers, median %-predicted FVC and DLCO were 70 (IQR 47-89) and 48 (IQR 32-74) respectively. 32% were on LTOT; median follow-up of 64 (IQR 20-120) months. Commonest diagnoses (83% of study group) were chILD category B1, 35.2% (sarcoidosis, hemosiderosis, connective tissue disorders, vasculitis); A4, 19% (surfactant-related); Bz, 14.6% (unclassified-ILD); and B2, 13.8% (bronchiolitis obliterans, hypersensitivity pneumonia). 60% were on systemic steroids. Not all chILD diagnoses were identified in adult-ILD classifications. <bold>Conclusion:</bold> The first ever documentation that chILD survivors represent a real burden of care in the adult-ILD centres due to their consistency though rare and their persisting-substantial physiologic impairment mandating ongoing monitoring and treatment. On the whole, the diagnosis of most chILD-survivors corresponded to similar ILD categories in the adult classification ensuring uninterrupted transition of specialist care. 17.3% of cases included non-ILD ultra-rare conditions; challenges may be encountered in identifying the appropriate receiving adult service. Discrepancies in taxonomy between chILD and adult-ILDs highlight a crucial need to harmonize current definitions to optimize clinical care and research.

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Published
2024-09-14
DOI
https://doi.org/10.1183/13993003.congress-2024.oa1066
Primary Topic
Adolescent and Pediatric Healthcare
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article
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article

Children’s ILDs survivors in adulthood: a European Study

C H Van Moorsel, Karlijn Groen, Rishi Pabary, Athanasios G. Kaditis et al.
Adolescent and Pediatric Healthcare
article

Children’s ILDs survivors in adulthood: a European Study

C H Van Moorsel, Karlijn Groen, Rishi Pabary, Athanasios G. Kaditis, Andriana Ι. Papaioannou, Elisabetta Renzoni, Ladislav Lacina, Hasan Yüksel, Alper Ezircan, Gökçen Kartal Öztürk, Alba Torrent‐Vernetta, Ebru Yalçın, Elisabeth Bendstrup, Halime Nayır Büyükşahin, Theofanis Tsiligiannis, Florence Jeny, Sedef Narin Tongal, Nagehan Emiralioğlu, Lampros Fotis, Y. Uzunhan, Antje Prasse, Rémi Diesler, Maria Kokosi, Thomas Skovhus Prior, Lykourgos Kolilekas, Şehnaz Olgun Yıldızeli, Cormac McCarthy, Antoine Froidure, Francesco Bonella, Helen O’Brien, Katarzyna Michel, Katerina Kourtesi, Yasemi̇n Gökdemi̇r, Nadia Nathan, Justyna Fijołek, Konstantinos Douros, Marie Legendre, Aurélie Fabre, Petr Pohunek, Camille Louvrier, María Molina‐Molina, Felix Chua, Nural Ki̇per, Francesca Mariani, Martina Vašáková, Ela Erdem Eralp, Matthias Griese, Nesrin Moğulkoç, Bulent Taner Karadag, Effrosyni D. Manali, Oğuz Karcıoğlu, Joanne J. van der Vis, Caroline Kannengiesser, Nesrin Öcal, Derya Kocakaya, Nicolaus Schwerk, Raphaël Borie, Maria Kallieri, Ilaria Campo, Elżbieta Radzikowska, Martina Safrankova, Demet Can, Andrew Bush, Hilario Nunes, Spyros Papiris, Vincent Cottin, Simon Chauveau, Bruno Crestani, Azrine Dahbia
article en

Abstract

<bold>Background:</bold> Children’s ILDs (chILD) often have a bad prognosis, but many survive into adulthood. <bold>Aim:</bold> To characterize chILD-survivors and identify their burden in adult-ILD centers. <bold>Methods:</bold> European study (33 adult-ILD and chILD centers) reporting incident/prevalent cases of chILD-survivors, January-July 2023. Epidemiological, clinical, physiologic, genetic data were collected. <bold>Results:</bold> Identified were 247 patients, median age at inclusion (IQR 25-75; years) of 25 (22-34), 50.2% male, 86% non-smokers, median %-predicted FVC and DLCO were 70 (IQR 47-89) and 48 (IQR 32-74) respectively. 32% were on LTOT; median follow-up of 64 (IQR 20-120) months. Commonest diagnoses (83% of study group) were chILD category B1, 35.2% (sarcoidosis, hemosiderosis, connective tissue disorders, vasculitis); A4, 19% (surfactant-related); Bz, 14.6% (unclassified-ILD); and B2, 13.8% (bronchiolitis obliterans, hypersensitivity pneumonia). 60% were on systemic steroids. Not all chILD diagnoses were identified in adult-ILD classifications. <bold>Conclusion:</bold> The first ever documentation that chILD survivors represent a real burden of care in the adult-ILD centres due to their consistency though rare and their persisting-substantial physiologic impairment mandating ongoing monitoring and treatment. On the whole, the diagnosis of most chILD-survivors corresponded to similar ILD categories in the adult classification ensuring uninterrupted transition of specialist care. 17.3% of cases included non-ILD ultra-rare conditions; challenges may be encountered in identifying the appropriate receiving adult service. Discrepancies in taxonomy between chILD and adult-ILDs highlight a crucial need to harmonize current definitions to optimize clinical care and research.

Université Claude Bernard Lyon 1 (FR), Izmir University (TR), Inserm (FR), Manisa Celal Bayar University (TR), National and Kapodistrian University of Athens (GR), Charles University (CZ), Royal Brompton & Harefield NHS Foundation Trust (GB), Bellvitge University Hospital (ES), Aarhus University Hospital (DK), Masih Daneshvari Hospital (IR), Medizinische Hochschule Hannover (DE), Sorbonne Université (FR), Ege University (TR), Hôpital Armand-Trousseau (FR), Institut d'Investigació Biomédica de Bellvitge (ES), Ruhrlandklinik (DE), Gülhane Askerî Tıp Akademisi (TR), Dr. Behçet Uz Çocuk Hastalıkları Hastanesi (TR), Assistance Publique – Hôpitaux de Paris (FR), University Hospital in Motol (CZ), Vall d'Hebron Institut de Recerca (ES), St. Antonius Ziekenhuis (NL), St. Vincent's University Hospital (IE), National Center for Tuberculosis and Lung Disease (GE), Children's Hospital Agia Sophia (GR), Vall d'Hebron Hospital Universitari (ES), Sağlık Bilimleri Üniversitesi (TR), Hôpital Avicenne (FR), German Center for Lung Research (DE), Mitera Hospital (GR), Hôpital Louis Pradel (FR), Thomayer University Hospital (CZ), Hôpital Bichat-Claude-Bernard (FR), Policlinico San Matteo Fondazione (IT), Sotiria General Hospital (GR), Maladies génétiques d’expression pédiatrique, Hacettepe University (TR), Marmara University (TR), Ludwig-Maximilians-Universität München (DE), UCLouvain (BE)
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Adolescent and Pediatric Healthcare
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