Childhood interstitial lung disease survivors in adulthood: a European collaborative study
Language English Country Great Britain, England Media electronic-print
Document type Journal Article, Multicenter Study
PubMed
39638417
DOI
10.1183/13993003.00680-2024
PII: 13993003.00680-2024
Knihovny.cz E-resources
- MeSH
- Child MeSH
- Adult MeSH
- Lung Diseases, Interstitial * epidemiology therapy physiopathology diagnosis MeSH
- Humans MeSH
- Adolescent MeSH
- Young Adult MeSH
- Survivors * statistics & numerical data MeSH
- Prognosis MeSH
- Check Tag
- Child MeSH
- Adult MeSH
- Humans MeSH
- Adolescent MeSH
- Young Adult MeSH
- Male MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Multicenter Study MeSH
- Geographicals
- Europe epidemiology MeSH
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.
7th Pulmonary Department Athens Chest Hospital Sotiria Athens Greece
Center for Biomedical Research on Rare Diseases Instituto de Salud Carlos 3 Barcelona Spain
Clinic for Pediatric Pneumology Allergology and Neonatology Hannover Medical School Hannover Germany
Contributed equally to this work
Département de Génétique APHP Hôpital Bichat Université de Paris Paris France
Department of Pulmonology Celal Bayar University Hospital Manisa Turkey
Department of Pulmonology Ege University Hospital Bornova Turkey
Department of Pulmonology Gulhane Faculty of Medicine Ankara Turkey
Department of Respiratory Diseases and Allergy Aarhus University Hospital Aarhus Denmark
Division of Adult Pulmonology Marmara University School of Medicine Istanbul Turkey
Division of Heart and Lungs University Medical Center Utrecht Utrecht The Netherlands
Division of Pediatric Pulmonology Marmara University School of Medicine Istanbul Turkey
Division of Pulmonology Department of Pediatrics Ege University Hospital Bornova Turkey
European Reference Network LUNG ILD Core Net Essen Germany
Hacettepe University Faculty of Medicine Ankara Turkey
ILD Clinic and Research Hannover Medical School Hannover Germany
Imperial Centre for Pediatrics and Child Health London UK
Interstitial Lung Disease Unit Guy's and St Thomas' Hospital NHS Foundation Trust London UK
Interstitial Lung Disease Unit Royal Brompton Hospital and Harefield NHS Foundation Trust London UK
Pediatric Pulmonology Hacettepe University Faculty of Medicine Ankara Turkey
Pediatric Pulmonology Mitera Pediatric Hospital Barcelona Spain
Pediatrics and Pediatrics Respirology Imperial College London London UK
Royal Brompton and Harefield Clinical Group Guy's and St Thomas' NHS Foundation Trust London UK
Royal Brompton Harefield NHS Foundation Trust London UK
Université de Paris UMR 1152 Inserm Paris France
University College Dublin School of Medicine St Vincent's University Hospital Dublin Ireland
Vall d'Hebron Institut de Recerca Hospital Universitari Vall d'Hebron Barcelona Spain
References provided by Crossref.org
An update on diagnosis and treatments of childhood interstitial lung diseases