Childhood interstitial lung disease survivors in adulthood: a European collaborative study
- PMID: 39638417
- DOI: 10.1183/13993003.00680-2024
Childhood interstitial lung disease survivors in adulthood: a European collaborative study
Abstract
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.
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Conflict of interest statement
Conflict of interest: E.D. Manali reports payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim, CSL Behring and Hoffman-La Roche; support for attending meetings and/or travel from Boehringer Ingelheim, CSL Behring, Hoffman-La Roche and Elpen; and leadership as Chair in the ERS task force for transition of chILD. M. Griese reports consulting fees and participation on a data safety monitoring board or advisory board with Boehringer Ingelheim. N. Nathan reports grants from CORTICONEHI for the clinical trial “Efficacy of methylprednisolone pulses in neuroendocrine cells hyperplasia of infancy”, the Million Dollar Bike Ride project for Neuroendocrine Cell Hyperplasia of Infancy, the Chancellerie des Universités, RespiFIL for the development of an e-learning module for CT-scan in childhood interstitial lung diseases and RespiFIL for the development of an online platform for rare lung disease quality of life and transition questionnaires; payment or honoraria for lectures, presentations, manuscript writing or educational events from La Lettre du Pneumologue; and support for attending meetings from the ERS. Y. Uzunhan reports grants from Oxyvie; consulting fees from Boehringer Ingelheim and Pfizer; payment or honoraria for lectures, presentations, manuscript writing or educational events from Sanofi, CSL Vifor and Boehringer Ingelheim; support for attending meetings and/or travel from Boehringer Ingelheim and Oxyvie; and participation on a data safety monitoring board or advisory board with Boehringer Ingelheim. R. Borie reports consulting fees from Boehringer Ingelheim, Ferrer and Sanofi; payment or honoraria for lectures, presentations; and educational events from Boehringer Ingelheim; and support for attending meetings from Boehringer Ingelheim. N. Schwerk reports consulting fees from Boehringer Ingelheim for consultancy work; paid lectures, publications and advisory board participation; and a leadership role as President-elect of the German Society for Paediatric Pneumology. C. McCarthy reports support for the present manuscript from Savara Inc.; grants from Health Research Board (Ireland), Enterprise Ireland and The LAM Foundation; consultancy fees from Savara Inc., AI Therapeutics, and Theravance Inc.; support for attending meetings from Boehringer Ingelheim; and participation on a data safety monitoring board or advisory board with Savara Inc. M. Koziar Vasakova reports consulting fees from Boehringer Ingelheim and Roche; payment or honoraria for lectures, presentations and educational events from Boehringer Ingelheim and Roche; support for attending meetings from Roche and Boehringer Ingelheim; and participation on a data safety monitoring board or advisory board with MSD and Boehringer Ingelheim. M. Molina-Molina reports grants from Boehringer Ingelheim and Roche; and payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim, Ferrer and Veracyte. C.H.M. van Moorsel reports grants from Boehringer Ingelheim for research support in digital auscultation for ILD, payment or honoraria for lectures from Boehringer Ingelheim and a leadership role as Co-chair of the ClinGen ILD Gene Curation Expert Panel. I. Campo reports consulting fees from Partner Therapeutics for a paid interview and participation on a data safety monitoring board or advisory board with Savara as a member of the advisory board for molgramostim. E. Bendstrup reports payment or honoraria for lectures, presentations, manuscript writing or educational events from AstraZeneca, Boehringer Ingelheim and Daichii Sankyo; support for attending meetings from Boehringer Ingelheim; and participation on a data safety monitoring board or advisory board with Simbec-Orion for Molecure DSMB. T.S. Prior reports payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim; and support for attending meetings from Boehringer Ingelheim. F. Bonella reports consulting fees from Boehringer Ingelheim, Sanofi, BMS and CSL-Behring; payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim and Sanofi; support for attending meetings from Boehringer Ingelheim, AstraZeneca and Atyr; and participation on a data safety monitoring board or advisory board with Boehringer Ingelheim, Sanofi and BMS. R. Diesler reports grants from CSL Behring, Fondation du Souffle and Fondation pour la Recherche Médicale, and support for attending meetings from Asdia. A. Froidure reports consulting fees from Boehringer Ingelheim; and payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim, AstraZeneca and GSK. F. Jeny reports payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim; and support for attending meetings from Oxyvie (travel for a French congress). N. Ocal reports payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim, Nobel and Humanis. B. Crestani reports grants from Boehringer Ingelheim; consultancy fees from BMS, Boehringer Ingelheim, Chiesi, GSK and Sanofi; payment or honoraria for lectures, presentations, manuscript writing or educational events from AstraZeneca, BMS, Boehringer Ingelheim, GSK, Novartis, Roche and Sanofi; support for attending meetings from AstraZeneca, BMS, Boehringer Ingelheim, Roche and Sanofi; participation on a data safety monitoring board or advisory board with BMS, Boehringer Ingelheim, Horizon and Sanofi; and a leadership role as President of the board of trustees of the Fondation du Souffle (a French charity). P. Pohunek reports support for the present study from the ERS Task Force TF2021-14 (payment for the librarian); consulting fees from AstraZeneca and GSK; payment or honoraria for lectures, presentations, manuscript writing or educational events from GSK and AstraZeneca (personal), and Chiesi, AstraZeneca and GSK (institutional); support for attending meetings from GSK and AstraZeneca; and participation on a data safety monitoring board or advisory board with GSK and AstraZeneca. S.A. Papiris reports payment or honoraria for lectures, presentations, manuscript writing or educational events from Boehringer Ingelheim and Hoffman La Roche; support for attending meetings from Boehringer Ingelheim, Hoffman La Roche and Elpen; and leadership or fiduciary role as a member in the ERS Task Force for the transition of chILD. The remaining authors have no potential conflicts of interest to disclose.