children<\/a> with rare interstitial lung conditions and suggest that standardised diagnostic criteria could support earlier identification of patients who may benefit from targeted treatment.\u00a0\u00a0<\/p>\nInvestigating Fibrosis in Childhood Lung Disease\u00a0<\/p>\n
Childhood interstitial lung disease encompasses a heterogeneous group of rare conditions affecting the lung tissue and surrounding structures.\u00a0<\/p>\n
Although pulmonary fibrosis is recognised as an important complication, its prevalence and relationship with long-term clinical outcomes have not previously been systematically established in large paediatric cohorts.\u00a0<\/p>\n
Researchers analysed data from the chILD-EU register, which includes children and adolescents from 23 European countries.\u00a0<\/p>\n
The prospective study examined 1,071 patients aged 0\u201318 years, with data collected between March 2004 and July 2025.\u00a0<\/p>\n
Researchers assessed pulmonary fibrosis using two definitions: predefined register criteria and a more restrictive definition used in clinical trials.\u00a0<\/p>\n
CT scans and lung biopsy findings underwent centralised peer review, while pulmonary function and survival were assessed longitudinally.\u00a0\u00a0<\/p>\n
One in Five Children Met Fibrosis Criteria\u00a0<\/p>\n
Of the 1,071 children included, 220 fulfilled the register definition of pulmonary fibrosis, corresponding to a prevalence of 20.5%.\u00a0<\/p>\n
Using the clinical trial definition, fibrosis was identified in 62 of 534 children, equivalent to 11.6%.\u00a0<\/p>\n
The median age at study inclusion was 2.4 years.\u00a0<\/p>\n
Children meeting either definition of pulmonary fibrosis had consistently poorer lung function than those without fibrosing disease.\u00a0<\/p>\n
Measured using percent predicted forced vital capacity, lung function was approximately 15\u201320% lower among children with fibrosis across follow-up visits extending to eight years.\u00a0<\/p>\n
These findings indicate that fibrosing disease is associated with a substantial and persistent reduction in respiratory function during childhood.\u00a0\u00a0<\/p>\n
Fibrosis Associated With Poorer Survival\u00a0<\/p>\n
Survival after enrolment was significantly lower among children meeting the register definition of pulmonary fibrosis.\u00a0<\/p>\n
The unadjusted hazard ratio for death or lung transplantation was 1.64 compared with children who did not fulfil the fibrosis criteria.\u00a0<\/p>\n
This association persisted after adjustment for sex, age category, and BMI z-score.\u00a0<\/p>\n
Although children meeting the clinical trial definition showed a similar pattern, the survival association did not reach statistical significance in that smaller group.\u00a0<\/p>\n
The researchers suggest that standardised fibrosis assessment could help clinicians identify children at increased risk of adverse outcomes and support more consistent selection of patients for future therapeutic trials.\u00a0<\/p>\n
However, the study was observational and did not evaluate whether antifibrotic treatment improves outcomes in children with chILD.\u00a0<\/p>\n
Further research is therefore required to establish the effectiveness and safety of antifibrotic therapies in paediatric populations.\u00a0<\/p>\n
The findings nevertheless demonstrate that pulmonary fibrosis is a clinically important feature of childhood interstitial lung disease, with implications for both respiratory function and survival.\u00a0\u00a0<\/p>\n
Reference\u00a0<\/p>\n
Griese M et al. Prevalence and disease trajectories of pulmonary fibrosis of childhood interstitial lung disease: a register-based, multicentre observational study. Lancet Respir Med. 2026;14(9):786\u2013796. doi:10.1016\/S2213-2600(26)00052-4.<\/p>\n","protected":false},"excerpt":{"rendered":"PULMONARY fibrosis affects approximately one in five children with childhood interstitial lung disease (chILD) and is associated with…\n","protected":false},"author":2,"featured_media":633071,"comment_status":"","ping_status":"","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[10],"tags":[163,85,46],"class_list":["post-633070","post","type-post","status-publish","format-standard","has-post-thumbnail","category-health","tag-health","tag-il","tag-israel"],"_links":{"self":[{"href":"https:\/\/www.newsbeep.com\/il\/wp-json\/wp\/v2\/posts\/633070","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/www.newsbeep.com\/il\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/www.newsbeep.com\/il\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/www.newsbeep.com\/il\/wp-json\/wp\/v2\/users\/2"}],"replies":[{"embeddable":true,"href":"https:\/\/www.newsbeep.com\/il\/wp-json\/wp\/v2\/comments?post=633070"}],"version-history":[{"count":0,"href":"https:\/\/www.newsbeep.com\/il\/wp-json\/wp\/v2\/posts\/633070\/revisions"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/www.newsbeep.com\/il\/wp-json\/wp\/v2\/media\/633071"}],"wp:attachment":[{"href":"https:\/\/www.newsbeep.com\/il\/wp-json\/wp\/v2\/media?parent=633070"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/www.newsbeep.com\/il\/wp-json\/wp\/v2\/categories?post=633070"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/www.newsbeep.com\/il\/wp-json\/wp\/v2\/tags?post=633070"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}