Pulmonary fibrosis affects approximately one in five children diagnosed with childhood interstitial lung disease, leaving patients with significantly impaired respiratory function and a higher risk of mortality or lung transplantation, according to a European multicentre study published in The Lancet Respiratory Medicine.
Registry Study Tracks 1,071 Pediatric Patients Across 23 European Countries
Childhood interstitial lung disease (chILD) encompasses a heterogeneous group of rare conditions affecting lung tissue and surrounding structures. To establish systematic data on how frequently pulmonary fibrosis develops in these young patients, researchers analyzed prospective data from the chILD-EU register, capturing 1,071 patients aged zero to 18 years between March 2004 and July 2025. The multicentre network involved contributions from 23 European countries, with contributions by DZL scientists Prof. Matthias Griese from CPC-M and PD Nicolaus Schwerk from BREATH. Expert panels performed centralised peer reviews of computed tomography scans and available lung biopsies to ensure uniform diagnostic standards.
Prevalence Rates Depend on Diagnostic Criteria Used
The study evaluated fibrosis using two distinct benchmarks: a predefined register definition and a stricter criteria set typically applied in clinical trials. Out of 1,071 children, 220 met the register definition, establishing a prevalence rate of 20.5 percent. Meanwhile, the restrictive clinical trial definition identified fibrosis in 62 out of 534 children, translating to 11.6 percent. The median age of patients at study inclusion was 2.4 years. Both definitions yielded consistent clinical trajectories, demonstrating that fibrotic lung remodelling occurs more frequently in paediatric populations than previously assumed.
Persistent Lung Function Reductions and Survival Impact
Children who met either fibrosis definition experienced persistent respiratory deficits throughout follow-up periods extending up to eight years. Measured via percent predicted forced vital capacity, lung function remained roughly 15 to 20 percent lower among fibrotic patients compared to those without fibrosing disease. Survival rates post-enrolment dropped significantly for children meeting the register definition. The unadjusted hazard ratio for death or the need for a lung transplantation reached 1.64 compared to children without fibrosis, an association that remained strong even after adjusting for sex, age category, and body mass index z-score. Interestingly, researchers noted that a higher body mass index correlated with a more favourable prognosis, matching observations seen in other chronic pulmonary conditions.
Clinical Implications and Treatment Horizons
The findings emphasize the clinical utility of adopting standardised diagnostic criteria to identify paediatric patients at elevated risk of adverse outcomes. While observational data clearly link fibrosis to worse outcomes, the study did not evaluate whether antifibrotic pharmacotherapy improves outcomes in children. Currently, only one antifibrotic medication holds approval for patients aged six and older, while other therapies remain largely restricted to adult care. Researchers stress that future clinical trials must rigorously test the safety and efficacy of these treatments in paediatric cohorts to bridge existing therapeutic gaps.
Worth a look