Idiopathic pulmonary fibrosis

FJ Martinez, HR Collard, A Pardo, G Raghu… - Nature reviews Disease …, 2017 - nature.com
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive lung disease characterized by
progressive lung scarring and the histological picture of usual interstitial pneumonia (UIP). It …

Idiopathic pulmonary fibrosis

L Richeldi, HR Collard, MG Jones - The Lancet, 2017 - thelancet.com
Idiopathic pulmonary fibrosis is a prototype of chronic, progressive, and fibrotic lung disease.
Healthy tissue is replaced by altered extracellular matrix and alveolar architecture is …

Therapeutic targets in lung tissue remodelling and fibrosis

G Liu, AM Philp, T Corte, MA Travis, H Schilter… - Pharmacology & …, 2021 - Elsevier
Structural changes involving tissue remodelling and fibrosis are major features of many
pulmonary diseases, including asthma, chronic obstructive pulmonary disease (COPD) and …

Targeting metabolic dysregulation for fibrosis therapy

X Zhao, JYY Kwan, K Yip, PP Liu, FF Liu - Nature reviews Drug …, 2020 - nature.com
Fibrosis is the abnormal deposition of extracellular matrix, which can lead to organ
dysfunction, morbidity, and death. The disease burden caused by fibrosis is substantial, and …

[HTML][HTML] Precision medicine advances in idiopathic pulmonary fibrosis

T Karampitsakos, BM Juan-Guardela, A Tzouvelekis… - …, 2023 - thelancet.com
Idiopathic pulmonary fibrosis (IPF) is a highly heterogeneous, unpredictable and ultimately
lethal chronic lung disease. Over the last decade, two anti-fibrotic agents have been shown …

The good and the bad collagens of fibrosis–their role in signaling and organ function

MA Karsdal, SH Nielsen, DJ Leeming… - Advanced drug delivery …, 2017 - Elsevier
Usually the dense extracellular structure in fibrotic tissues is described as extracellular
matrix (ECM) or simply as collagen. However, fibrosis is not just fibrosis, which is already …

[HTML][HTML] Idiopathic pulmonary fibrosis (IPF): an overview

SL Barratt, A Creamer, C Hayton… - Journal of clinical …, 2018 - mdpi.com
Idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease characterised by chronic,
progressive scarring of the lungs and the pathological hallmark of usual interstitial …

The therapy of idiopathic pulmonary fibrosis: what is next?

V Somogyi, N Chaudhuri, SE Torrisi… - European …, 2019 - Eur Respiratory Soc
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrosing interstitial lung
disease, characterised by progressive scarring of the lung and associated with a high …

[HTML][HTML] The natural history of progressive fibrosing interstitial lung diseases

M Kolb, M Vašáková - Respiratory research, 2019 - Springer
A proportion of patients with certain types of interstitial lung disease (ILD), including chronic
hypersensitivity pneumonitis and ILDs associated with autoimmune diseases, develop a …

Extracellular matrix in lung development, homeostasis and disease

Y Zhou, JC Horowitz, A Naba, N Ambalavanan… - Matrix Biology, 2018 - Elsevier
The lung's unique extracellular matrix (ECM), while providing structural support for cells, is
critical in the regulation of developmental organogenesis, homeostasis and injury-repair …