Time for a change: is idiopathic pulmonary fibrosis still idiopathic and only fibrotic?

PJ Wolters, TS Blackwell, O Eickelberg… - The Lancet …, 2018 - thelancet.com
Idiopathic pulmonary fibrosis (IPF) is a progressive, irreversible, and typically fatal lung
disease characterised by subpleural fibrosis, subepithelial fibroblast foci, and microscopic …

Angiotensin-TGF-β1 crosstalk in human idiopathic pulmonary fibrosis: autocrine mechanisms in myofibroblasts and macrophages

BD Uhal, J Kyong Kim, X Li… - Current pharmaceutical …, 2007 - ingentaconnect.com
Angiotensin II (ANGII) has been identified as a proapoptotic and profibrotic factor in
experimental lung fibrosis models, and patients with the ID/DD polymorphism of ANG …

Angiotensin signalling in pulmonary fibrosis

BD Uhal, X Li, CC Piasecki, M Molina-Molina - The international journal of …, 2012 - Elsevier
A large body of evidence demonstrates that angiotensin II and angiotensin receptors are
required for the pathogenesis of experimental lung fibrosis. Angiotensin has a number of …

Idiopathic pulmonary fibrosis (an update) and progressive pulmonary fibrosis in adults: an official ATS/ERS/JRS/ALAT clinical practice guideline

G Raghu, M Remy-Jardin, L Richeldi… - American Journal of …, 2022 - atsjournals.org
Background: This American Thoracic Society, European Respiratory Society, Japanese
Respiratory Society, and Asociación Latinoamericana de Tórax guideline updates prior …

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 …

Outcome of hospitalization for COVID-19 in patients with interstitial lung disease. An international multicenter study

TM Drake, AB Docherty, EM Harrison… - American journal of …, 2020 - atsjournals.org
Rationale: The impact of coronavirus disease (COVID-19) on patients with interstitial lung
disease (ILD) has not been established. Objectives: To assess outcomes in patients with ILD …

Genome-wide association study of susceptibility to idiopathic pulmonary fibrosis

RJ Allen, B Guillen-Guio, JM Oldham… - American journal of …, 2020 - atsjournals.org
Rationale: Idiopathic pulmonary fibrosis (IPF) is a complex lung disease characterized by
scarring of the lung that is believed to result from an atypical response to injury of the …

Angiotensin converting enzyme-2 is protective but downregulated in human and experimental lung fibrosis

X Li, M Molina-Molina, A Abdul-Hafez… - … of Physiology-Lung …, 2008 - journals.physiology.org
Earlier work from this laboratory showed that local generation of angiotensin (ANG) II is
required for the pathogenesis of experimental pulmonary fibrosis and that ANG peptides are …

Ziritaxestat, a novel autotaxin inhibitor, and lung function in idiopathic pulmonary fibrosis: the ISABELA 1 and 2 randomized clinical trials

TM Maher, P Ford, KK Brown, U Costabel, V Cottin… - Jama, 2023 - jamanetwork.com
Importance There is a major need for effective, well-tolerated treatments for idiopathic
pulmonary fibrosis (IPF). Objective To assess the efficacy and safety of the autotaxin inhibitor …

[HTML][HTML] The JAK2 pathway is activated in idiopathic pulmonary fibrosis

J Milara, G Hernandez, B Ballester, A Morell, I Roger… - Respiratory …, 2018 - Springer
Background Idiopathic pulmonary fibrosis (IPF) is the most rapidly progressive and fatal
fibrotic disorder, with no curative therapies. The signal transducer and activator of …