Prevalence and disease trajectories of pulmonary fibrosis of childhood interstitial lung disease: a register-based, multicentre observational study
Rattachement africain : de, ch, pl, dk, be, tr, es, gb. Niveau de preuve : code pays fourni par la source.
Le résumé fourni par la source
Background Pulmonary fibrosis is of critical importance in childhood interstitial lung disease (chILD), yet fibrosis prevalence, impact on clinical progression, and survival have not been systematically evaluated. Therefore, we aimed to determine the prevalence of pulmonary fibrosis and its impact on lung function as well as survival in individuals aged 0–18 years. Methods Data were extracted from the chILD-EU register, a multicentre cohort study of children and adolescents with chILD from 23 European countries. Data collection included centralised peer review and systematic scoring of CT scans and lung biopsies. The primary outcome was the presence or absence of fibrosing lung disease. Pulmonary fibrosis was determined based on predefined criteria used in the register (fibrosis register ) or in clinical trials (fibrosis trial ). Clinical characteristics, disease categories, pulmonary function data, and survival were assessed. This study was registered with ClinicalTrials.gov (NCT02852928) and is ongoing. Findings Data were collected prospectively between March 3, 2004 and July 23, 2025. Among the 1071 children diagnosed with chILD, 220 of 1071 fulfilled the criteria of fibrosis register (20·5% [95% CI 18·1–23·0]) and 62 of 534 fulfilled the criteria of fibrosis trial (11·6% [8·9–14·3]). Median age at inclusion was 2·4 years (IQR 0·6–9·2), 570 (53·2%) participants were male and 501 (46·8%) were female, and 882 (76·8%) were European. Pulmonary function, assessed as percent predicted forced vital capacity (ppFVC), in children with fibrosis under both fibrosis definitions was consistently 15–20% lower across all follow-up visits up to 8 years compared with individuals without fibrosing lung disease. Survival after enrolment was lower in children who fulfilled fibrosis register criteria than in those who did not fulfil fibrosis register criteria (log-rank p=0·0029; unadjusted hazard ratio (HR) for death or lung transplantation 1·64 [95% CI 1·18–2·28]). The increased hazard of fibrosis register remained after adjusting for sex, age category, and BMI z-score. A higher BMI z-score was protective for survival with fibrosis register (HR 0·80). While not statistically significant, children with fibrosis trial criteria had survival and HR trends in the same direction as those with fibrosis register criteria. Interpretation The application of standardised criteria for diagnosing pulmonary fibrosis enables identification of affected children among patients with chILD. These children have lower pulmonary function, an increased risk of death, and could benefit from antifibrotic therapies. Funding Deutsche Forschungsgemeinschaft and Boehringer Ingelheim, Germany.
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Le contrôle bibliographique ouvert
DOI retrouvé dans Crossref DOI retrouvé ; titre concordant.
- Titre Crossref
- Prevalence and disease trajectories of pulmonary fibrosis of childhood interstitial lung disease: a register-based, multicentre observational study
- Date Crossref
- 01/09/2026
- Éditeur
- Elsevier BV
- Type
- journal-article
Ce recoupement confirme des métadonnées liées au DOI. Il ne confirme ni la méthode ni les conclusions de l’étude, et il ne compte pas comme une seconde source scientifique indépendante.
Où se fait cette recherche
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German Center for Lung Research pays non établi dans la noticeStructure de recherche
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Ludwig-Maximilians-Universität München pays non établi dans la noticeUniversité ou école supérieure
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University of Würzburg Institute of Pathology pays non établi dans la noticeUniversité ou école supérieure
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LMU Klinikum pays non établi dans la noticeÉtablissement de santé
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University Children's Hospital Zurich Department of Respiratory Medicine pays non établi dans la noticeÉtablissement de santé
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Medizinische Hochschule Hannover pays non établi dans la noticeUniversité ou école supérieure
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Medical University of Warsaw Department of Pediatric Pneumology and Allergy pays non établi dans la noticeUniversité ou école supérieure
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Copenhagen University Hospital Department of Pediatrics pays non établi dans la noticeÉtablissement de santé
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Rigshospitalet pays non établi dans la noticeÉtablissement de santé
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Zentrum für Kinderheilkunde pays non établi dans la noticeÉtablissement de santé
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University of Duisburg-Essen pays non établi dans la noticeUniversité ou école supérieure
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Goethe University Frankfurt pays non établi dans la noticeUniversité ou école supérieure
German Center for Lung Research, Ludwig-Maximilians-Universität München et Institute of Pathology — University of Würzburg, avec 9 autres affiliations.
Une affiliation ne permet pas de déduire la nationalité d’un auteur.