Insight into the pathogenesis of interstitial lung diseases and
near-to-native lung fibrosis models

J 2025

Insight into the pathogenesis of interstitial lung diseases and near-to-native lung fibrosis models

ŠTEFÁNIKOVÁ, Marianna; Veronika SEDLÁKOVÁ; Türkan PORTAKAL; Aleš HAMPL; Petr VAŇHARA et al.

Základní údaje

Originální název

Insight into the pathogenesis of interstitial lung diseases and near-to-native lung fibrosis models

Autoři

ŠTEFÁNIKOVÁ, Marianna ; Veronika SEDLÁKOVÁ; Türkan PORTAKAL; Aleš HAMPL; Petr VAŇHARA a Martina DOUBKOVÁ

Vydání

European Journal of Medical Research, London, Biomed Central, 2025, 0949-2321

Další údaje

Jazyk

angličtina

Typ výsledku

Článek v odborném periodiku

Obor

30203 Respiratory systems

Stát vydavatele

Velká Británie a Severní Irsko

Utajení

není předmětem státního či obchodního tajemství

Odkazy

URL

Impakt faktor

Impact factor: 3.400 v roce 2024

Označené pro přenos do RIV

Ano

Kód RIV

RIV/00216224:14110/25:00143765

Organizační jednotka

Lékařská fakulta

Klíčová slova anglicky

Interstitial lung disease; Pathogenesis; Lung fibrosis models; Organoids

Štítky

rivok

Příznaky

Mezinárodní význam, Recenzováno

Změněno: 24. 2. 2026 10:43, Mgr. Tereza Miškechová

Anotace

V originále

Interstitial lung diseases (ILDs) is a large and heterogeneous group of disorders with a variable degree of lung inflammation and lung fibrosis. In some ILDs, we can observe a progressive-fibrosing phenotype-PF-ILD (e.g., idiopathic pulmonary fibrosis, fibrotic phenotype of hypersensitivity pneumonitis, familial lung fibrosis, etc.). Lung fibrosis is characterized by overgrowth, stiffening, and scarring of tissues due to excess deposition of extracellular matrix. In some patients suffering from PF-ILD, progression and fatal outcomes occur despite treatment. Therefore, there is a great need for the development of lung fibrosis models that will help to understand and recapitulate the etiopathogenesis of the disease and may thus serve as tools for unraveling its underlying profibrotic mechanisms and potential therapeutic targets. In this review, we summarize ILD etiopathogenesis, current and novel therapeutic options, and discuss in vivo, ex vivo, and in vitro near-to-native lung fibrosis models, which help to elucidate specific processes within ILD pathophysiology.

Návaznosti

GA23-06675S, projekt VaV

Název: Plicní stres a regenerace

Investor: Grantová agentura ČR, Plicní stres a regenerace

MUNI/A/1738/2024, interní kód MU

Název: Zdroje pro tkáňové inženýrství 15

Investor: Masarykova univerzita, Zdroje pro tkáňové inženýrství 15

Citovat

ŠTEFÁNIKOVÁ, Marianna; Veronika SEDLÁKOVÁ; Türkan PORTAKAL; Aleš HAMPL; Petr VAŇHARA a Martina DOUBKOVÁ. Insight into the pathogenesis of interstitial lung diseases and near-to-native lung fibrosis models. European Journal of Medical Research. London: Biomed Central, 2025, roč. 30, č. 1, s. 1-21. ISSN 0949-2321. Dostupné z: https://doi.org/10.1186/s40001-025-03504-4.

@article{2556397,
   author = {Štefániková, Marianna and Sedláková, Veronika and Portakal, Türkan and Hampl, Aleš and Vaňhara, Petr and Doubková, Martina},
   article_location = {London},
   article_number = {1},
   doi = {https://doi.org/10.1186/s40001-025-03504-4},
   keywords = {Interstitial lung disease; Pathogenesis; Lung fibrosis models; Organoids},
   language = {eng},
   issn = {0949-2321},
   journal = {European Journal of Medical Research},
   title = {Insight into the pathogenesis of interstitial lung diseases and near-to-native lung fibrosis models},
   url = {https://link.springer.com/article/10.1186/s40001-025-03504-4},
   volume = {30},
   year = {2025}
}

TY  - JOUR
ID  - 2556397
AU  - Štefániková, Marianna - Sedláková, Veronika - Portakal, Türkan - Hampl, Aleš - Vaňhara, Petr - Doubková, Martina
PY  - 2025
TI  - Insight into the pathogenesis of interstitial lung diseases and near-to-native lung fibrosis models
JF  - European Journal of Medical Research
VL  - 30
IS  - 1
SP  - 1-21
EP  - 1-21
PB  - Biomed Central
SN  - 09492321
KW  - Interstitial lung disease
KW  - Pathogenesis
KW  - Lung fibrosis models
KW  - Organoids
UR  - https://link.springer.com/article/10.1186/s40001-025-03504-4
N2  - Interstitial lung diseases (ILDs) is a large and heterogeneous group of disorders with a variable degree of lung inflammation and lung fibrosis. In some ILDs, we can observe a progressive-fibrosing phenotype-PF-ILD (e.g., idiopathic pulmonary fibrosis, fibrotic phenotype of hypersensitivity pneumonitis, familial lung fibrosis, etc.). Lung fibrosis is characterized by overgrowth, stiffening, and scarring of tissues due to excess deposition of extracellular matrix. In some patients suffering from PF-ILD, progression and fatal outcomes occur despite treatment. Therefore, there is a great need for the development of lung fibrosis models that will help to understand and recapitulate the etiopathogenesis of the disease and may thus serve as tools for unraveling its underlying profibrotic mechanisms and potential therapeutic targets. In this review, we summarize ILD etiopathogenesis, current and novel therapeutic options, and discuss in vivo, ex vivo, and in vitro near-to-native lung fibrosis models, which help to elucidate specific processes within ILD pathophysiology.
ER  -

ŠTEFÁNIKOVÁ, Marianna; Veronika SEDLÁKOVÁ; Türkan PORTAKAL; Aleš HAMPL; Petr VAŇHARA a Martina DOUBKOVÁ. Insight into the pathogenesis of interstitial lung diseases and near-to-native lung fibrosis models. \textit{European Journal of Medical Research}. London: Biomed Central, 2025, roč.~30, č.~1, s.~1-21. ISSN~0949-2321. Dostupné z: https://doi.org/10.1186/s40001-025-03504-4.

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