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.

1st Department of Paediatrics Agia Sophia Children's Hospital Medical School National and Kapodistrian University of Athens Athens Greece

1st University Department of Respiratory Medicine National and Kapodistrian University of Athens Athens Greece

2nd Pulmonary Medicine Department General University Hospital Attikon Medical School National and Kapodistrian University of Athens Athens Greece

3rd Department of Lung Diseases and Oncology National Tuberculosis and Lung Diseases Research Institute Warsaw Poland

3rd Department of Paediatrics General University Hospital Attikon Medical School National and Kapodistrian University of Athens Athens Greece

7th Pulmonary Department Athens Chest Hospital Sotiria Athens Greece

APHP Hôpital Bichat Service de Pneumologie A FHU APOLLO Centre de Référence des Maladies Pulmonaires Rares Paris France

Center for Biomedical Research on Rare Diseases Instituto de Salud Carlos 3 Barcelona Spain

Center for Interstitial and Rare Lung Diseases Pneumology Department Ruhrlandklinik University Hospital University of Essen Essen Germany

Centre de Référence Constitutif des Maladies Pulmonaires Rares Service de Pneumologie Hôpital Avicenne APHP Bobigny France

Childhood Genetic Diseases Laboratory UMR S933 Inserm Sorbonne Université Hôpital Armand Trousseau Paris France

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 Paediatrics Obstetrics Gynecology Preventative Medicine and Public Health Universitat Autònoma de Barcelona Bellaterra Spain

Department of Pediatric Pneumology Dr von Hauner Children's Hospital Ludwig Maximilians University German Center for Lung Research Munich Germany

Department of Pediatrics 2nd Faculty of Medicine Charles University and University Hospital Motol Prague Czech Republic

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

Department of Respiratory Medicine 1st Faculty of Medicine Charles University and Thomayer University Hospital Thomayer University Hospital Prague Czech Republic

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 Allergy and Clinical Immunology Dr BehcetUz Children's Hospital University of Health Sciences Izmir Turkey

Division of Pediatric Pulmonology Marmara University School of Medicine Istanbul Turkey

Division of Pulmonology Department of Pediatrics Ege University Hospital Bornova Turkey

ERN Lung Interstitial Lung Disease Center of Excellence Department of Pulmonology St Antonius Hospital Nieuwegein The Netherlands

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

Laboratorio di Biochimica e Genetica U O C Pneumologia Fondazione IRCCS Policlinico San Matteo viale Golgi Pavia Italy

Margaret Turner Warwick Centre for Fibrosing Lung Disease National Heart and Lung Institute Imperial College London London UK

Multidisciplinary Unit Hospital Universitari de Bellvitge IDIBELL L'Hospitalet del Llobreg Barcelona Spain

Paediatric Allergy and Pulmonology Section Department of Paediatrics Vall d'Hebron Hospital Universitari Vall d'Hebron Barcelona Hospital Campus Universitat Autònoma de Barcelona Barcelona Spain

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

Pulmonology Department Cliniques Universitaires Saint Luc Université Catholique de Louvain Brussels Institut de Recherche Expérimentale et Clinique Brussels Belgium

Royal Brompton and Harefield Clinical Group Guy's and St Thomas' NHS Foundation Trust London UK

Royal Brompton Harefield NHS Foundation Trust London UK

Service de pneumologie Centre national coordinateur de référence des maladies pulmonaires rares Hôpital Louis Pradel Hospices Civils de Lyon Université de Lyon Université Claude Bernard Lyon 1 UMR754 INRA IVPC Lyon France

Service de Pneumologie Pédiatrique Assistance Publique Hôpitaux de Paris Sorbonne Université Centre National de Référence des Maladies Respiratoires Rares RespiRare Hôpital Armand Trousseau Paris France

Université de Paris UMR 1152 Inserm Paris France

University College Dublin School of Medicine Education and Research Centre St Vincent's University Hospital Dublin Ireland

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

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An update on diagnosis and treatments of childhood interstitial lung diseases