Prevalence and disease trajectories of pulmonary fibrosis of childhood interstitial lung disease: a register-based, multicentre observational study.
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Griese Matthias, Reu-Hofer Simone, Ley-Zaporozhan Julia, Kammer Birgit, Krüger-Stollfuß Ingrid, Micic Srdjan, Carlens Julia, Maier Pia, Rodler Julia, Mauss-Schwarzer Katharina, Tran Nguyen-Binh, Rapp Christina Katharina, Gothe Florian, Marczak Honorata, Lange Joanna, Krenke Katarzyna, Ring Astrid Madsen, Buchvald Frederik, Stehling Florian, Jerkic Pera Silvija, Trischler Jordis, Proesmans Marijke, Sismanlar Tugba, Aslan Ayse, Emiralioğlu Nagehan, Kiper Nural, Hämmerling Susanne, Kılınç Ayşe, Prenzel Freerk, Wiemers Anna, Moreno-Galdo Antonio, Mayell Sarah, Bhatt Jayesh Mahendra, Naehrlich Lutz, Pahlke Friedrich, Wetzke Martin, Moeller Alexander, Kappler Matthias, chILD-EU collaborators, Schwerk Nicolaus, Seidl Elias
Аннотация
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 (fibrosisregister) or in clinical trials (fibrosistrial). 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 fibrosisregister (20·5% [95% CI 18·1-23·0]) and 62 of 534 fulfilled the criteria of fibrosistrial (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 fibrosisregister criteria than in those who did not fulfil fibrosisregister 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 fibrosisregister remained after adjusting for sex, age category, and BMI z-score. A higher BMI z-score was protective for survival with fibrosisregister (HR 0·80). While not statistically significant, children with fibrosistrial criteria had survival and HR trends in the same direction as those with fibrosisregister 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.