Shared and distinct mechanisms of fibrosis

JHW Distler, AH Györfi, M Ramanujam… - Nature Reviews …, 2019 - nature.com
Fibrosis is defined as an excessive deposition of connective tissue components and can
affect virtually every organ system, including the skin, lungs, liver and kidney. Fibrotic tissue …

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 …

[HTML][HTML] Inflammation and immunity in IPF pathogenesis and treatment

P Heukels, CC Moor, JH Von der Thüsen… - Respiratory …, 2019 - Elsevier
Idiopathic pulmonary fibrosis (IPF) is a progressive, and ultimately fatal, chronic interstitial
lung disease characterized by enhanced extracellular matrix deposition. Repetitive alveolar …

[HTML][HTML] Antifibrotic therapy for idiopathic pulmonary fibrosis: time to treat

TM Maher, ME Strek - Respiratory research, 2019 - Springer
Idiopathic pulmonary fibrosis (IPF) is a progressive disease with a dismal prognosis. The
average life expectancy of untreated patients with IPF is only 3 to 4 years. Decline in forced …

[HTML][HTML] Interstitial lung disease associated with systemic sclerosis (SSc-ILD)

V Cottin, KK Brown - Respiratory research, 2019 - Springer
Abstract Background Systemic sclerosis (SSc) is a rare connective tissue disease with a
heterogeneous clinical course. Interstitial lung disease (ILD) is a common manifestation of …

[HTML][HTML] Targeting oxidative stress as a therapeutic approach for idiopathic pulmonary fibrosis

C Estornut, J Milara, MA Bayarri, N Belhadj… - Frontiers in …, 2022 - frontiersin.org
Idiopathic pulmonary fibrosis (IPF) is a chronic interstitial lung disease characterized by an
abnormal reepithelialisation, an excessive tissue remodelling and a progressive fibrosis …

Pirfenidone: molecular mechanisms and potential clinical applications in lung disease

SM Ruwanpura, BJ Thomas… - American journal of …, 2020 - atsjournals.org
Pirfenidone (PFD) is a pharmacological compound with therapeutic efficacy in idiopathic
pulmonary fibrosis. It has been chiefly characterized as an antifibrotic agent, although it was …

Nintedanib with add-on pirfenidone in idiopathic pulmonary fibrosis. Results of the INJOURNEY trial

C Vancheri, M Kreuter, L Richeldi… - American journal of …, 2018 - atsjournals.org
Rationale: Nintedanib and pirfenidone slow the progression of idiopathic pulmonary fibrosis
(IPF), but the disease continues to progress. More data are needed on the safety and …

Immune mechanisms in pulmonary fibrosis

S Kolahian, IE Fernandez, O Eickelberg… - American journal of …, 2016 - atsjournals.org
Pulmonary fibrosis, particularly idiopathic pulmonary fibrosis, represents a chronic and
progressive disease with high mortality and limited therapeutic options. Excessive …

The myofibroblast, a key cell in normal and pathological tissue repair

IA Darby, N Zakuan, F Billet, A Desmoulière - Cellular and Molecular Life …, 2016 - Springer
Myofibroblasts are characterized by their expression of α-smooth muscle actin, their
enhanced contractility when compared to normal fibroblasts and their increased synthetic …