Menu

Latest Information

2025-01-23

Pulmonary Fibrosis – A story from childhood to adult survivors

News 2025-044 EN

Childhood interstitial lung disease (childhood ILD = chILD) is a group of rare, diffuse respiratory diseases in children. Depending on the underlying genetic variations, patients have a poor prognosis with substantial mortality. However, the group of children and adolescents with chILD who reach adulthood is growing. How are these patients doing? What are the problems in this orphaned group and how are the patients coping with their disease? How successful is the transition from care in pediatric clinics into adult services?

Two scientific projects in which DZL-PI Matthias Griese (Dr von Haunersches children hospital, DZL site CPC-M) played a key role provide interesting answers to these questions. As the first author of the study “Pulmonary fibrosis may begin in infancy: from childhood to adult interstitial lung disease” (Thorax, 2024), he showed: There is a lot of catching up to do, in particular the transition from childhood interstitial lung disease to adult disease needs to be clarified and to be incorporated into appropriate medical guidelines.

Over the last decade, the spectrum of conditions within chILD has widened substantially. However, most evidence is often limited to small case series, with reports disseminated across an array of severe subspecialties, clinical and molecular journals. In particular, the frequency, management and outcome of pediatric pulmonary fibrosis is not well characterized, unlike in adults, where clear diagnosis and treatment guidelines are available.

The paper in Thorax assessed the current understanding of pulmonary fibrosis in chILD. Based on registry data, the occurrence of pulmonary fibrosis in was identified in 47 different disease entities.

As coordinator of the European Network for Rare Lung Diseases (chILD-EU), Griese wanted to go one step further: In a study in the European Respiratory Journal, he and other European scientists examined for the first time how former chILD patients grew up and become adult pulmonary fibrosis patients. The team's findings: When patients transitioned from pediatric to adult specialty care, diagnoses corresponded mostly, but not always to similar ILD categories in the adult classification systems. This may lead to potentially inappropriate diagnostic labeling. The researchers are convinced that the effects of this suboptimal transition to adult ILD need to be further investigated. Re-specification of diagnosis and targeted treatments could contribute to precision and personalization. Finally, we are in need of updated evidence-based guidelines that include recommendations for the diagnosis and treatment of interstitial lung diseases in children and their appropriate transition into adult pulmonology.

Original publications:

Griese M, Kurland G, Cidon M, Deterding RR, Epaud R, Nathan N, Schwerk N, Warburton D, Weinman JP, Young LR, Deutsch GH. Pulmonary fibrosis may begin in infancy: from childhood to adult interstitial lung disease. Thorax. 2024 Nov 14;79(12):1162-1172. doi: 10.1136/thorax-2024-221772. PMID: 39153860; PMCID: PMC11671978.

Manali ED, Griese M, Nathan N, Uzunhan Y, Borie R, Michel K, Schwerk N, Fijolek J, Radzikowska E, Chua F, Pabary R, Mogulkoc N, McCarthy C, Kallieri M, Papaioannou AI, Kiper N, Koziar Vasakova M, Lacina L, Molina-Molina M, Torrent-Vernetta A, Tsiligiannis T, Karadag B, Kokosi M, Renzoni EA, Hm van Moorsel C, Campo I, Bendstrup E, Skovhus Prior T, Prasse A, Bonella F, Cottin V, Diesler R, Froidure A, Kolilekas L, Fotis L, Douros K, Kaditis AG, Jeny F, Chauveau S, Nunes H, Dahbia A, Mariani F, van der Vis JJ, Groen K, Erdem Eralp E, Gokdemir Y, Kocakaya D, Olgun Yildizeli S, Yalçın E, Emiralioğlu N, Nayir Buyuksahin H, O'Brien H, Karcıoglu O, Can D, Ezircan A, Kartal Ozturk G, Ocal N, Yuksel H, Narin Tongal S, Safrankova M, Kourtesi K, Louvrier C, Kannengiesser C, Fabre A, Legendre M, Crestani B, Pohunek P, Bush A, Papiris SA. Childhood interstitial lung disease survivors in adulthood: a European collaborative study. Eur Respir J. 2024 Dec 5:2400680. doi: 10.1183/13993003.00680-2024. Epub ahead of print. PMID: 39638417.

Further Information:

https://www.childeu.net/childeu

https://www.ccrc-hauner.de/research-labs/griese-lab/26d5c8e071fb6319

DZL Engagements

chevron-down