[HTML][HTML] Targeting fibrosis: Mechanisms and clinical trials

M Zhao, L Wang, M Wang, S Zhou, Y Lu… - Signal transduction and …, 2022 - nature.com
Fibrosis is characterized by the excessive extracellular matrix deposition due to
dysregulated wound and connective tissue repair response. Multiple organs can develop …

Idiopathic pulmonary fibrosis: Disease mechanisms and drug development

P Spagnolo, JA Kropski, MG Jones, JS Lee… - Pharmacology & …, 2021 - Elsevier
Idiopathic pulmonary fibrosis (IPF) is a chronic progressive disease of unknown cause
characterized by relentless scarring of the lung parenchyma leading to reduced quality of life …

Pirfenidone in patients with progressive fibrotic interstitial lung diseases other than idiopathic pulmonary fibrosis (RELIEF): a double-blind, randomised, placebo …

J Behr, A Prasse, M Kreuter, J Johow… - The Lancet …, 2021 - thelancet.com
Background Pirfenidone has been shown to slow disease progression in patients with
idiopathic pulmonary fibrosis (IPF). However, there are few treatment options for progressive …

Pirfenidone in patients with unclassifiable progressive fibrosing interstitial lung disease: a double-blind, randomised, placebo-controlled, phase 2 trial

TM Maher, TJ Corte, A Fischer, M Kreuter… - The lancet Respiratory …, 2020 - thelancet.com
Background At present, no approved pharmacotherapies are available for unclassifiable
interstitial lung disease (ILD), which is characterised by progressive fibrosis of the lung. We …

Idiopathic pulmonary fibrosis: Current and future treatment

DS Glass, D Grossfeld, HA Renna… - The clinical …, 2022 - Wiley Online Library
Objectives Idiopathic pulmonary fibrosis (IPF) is a chronic fibrotic lung disease characterized
by dry cough, fatigue, and progressive exertional dyspnea. Lung parenchyma and …

Progressive fibrosing interstitial lung disease: clinical uncertainties, consensus recommendations, and research priorities

PM George, P Spagnolo, M Kreuter… - The lancet Respiratory …, 2020 - thelancet.com
Within the spectrum of fibrosing interstitial lung diseases (ILDs) is a subset of patients who
have inexorable progression of pulmonary fibrosis despite treatment, which is known as the …

Pirfenidone in heart failure with preserved ejection fraction: a randomized phase 2 trial

GA Lewis, S Dodd, D Clayton, E Bedson, H Eccleson… - Nature Medicine, 2021 - nature.com
In heart failure with preserved ejection fraction (HFpEF), the occurrence of myocardial
fibrosis is associated with adverse outcome. Whether pirfenidone, an oral antifibrotic agent …

TGF-β1–a truly transforming growth factor in fibrosis and immunity

M Lodyga, B Hinz - Seminars in cell & developmental biology, 2020 - Elsevier
Abstract 'Jack of all trades, master of everything'is a fair label for transforming growth factor
β1 (TGF-β)–a cytokine that controls our life at many levels. In the adult organism, TGF-β1 is …

[HTML][HTML] Alveolar epithelial type II cells as drivers of lung fibrosis in idiopathic pulmonary fibrosis

T Parimon, C Yao, BR Stripp, PW Noble… - International journal of …, 2020 - mdpi.com
Alveolar epithelial type II cells (AT2) are a heterogeneous population that have critical
secretory and regenerative roles in the alveolus to maintain lung homeostasis. However …

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 …