Idiopathic Pulmonary Fibrosis: An Update on Pathogenesis
WEBJan 19, 2022 · Histopathological characteristics of IPF include excessive deposition of the extracellular matrix (ECM), leading to distortion of normal lung architecture and irreversible loss of lung function (Glass et al., 2020). IPF is clinically manifested by progressive dyspnea and a significant decrease in lung compliance (Schafer et al., 2020).
Extracellular matrix as a driver of progressive fibrosis - PMC
WEBJan 2, 2018 · However, studies of idiopathic pulmonary fibrosis (IPF), a relentlessly progressive disorder, have revealed mechanisms of fibrosis progression that can be self-sustaining once established (8, 9). This indicates that …
Oxidative stress, extracellular matrix targets, and idiopathic ...
WEBSep 1, 2010 · Markers of oxidative stress have been identified in the lungs of IPF patients and aberrant antioxidant activity exacerbates pulmonary fibrosis in animal models. In addition, the extracellular matrix is a critical component in regulating cellular homeostasis and appropriate wound healing.
Idiopathic pulmonary fibrosis: Current and future treatment
WEBJan 10, 2022 · Etiopathogenesis of IPF is not fully understood, and treatment options are limited. Pathological features of IPF include extracellular matrix remodeling, fibroblast activation and proliferation, immune dysregulation, cell senescence, and presence of aberrant basaloid cells.
Extracellular Matrix Remodeling in Idiopathic Pulmonary Fibrosis
WEBIdiopathic pulmonary fibrosis (IPF) is a progressive and lethal lung disorder characterized by fibroblast proliferation and extracellular matrix accumulation. The molecular mechanisms behind the aberrant tissue remodeling should involve the families of matrix metalloproteinases (MMPs) and tissue inhibitor of metalloproteinases (TIMPs), main ...
Oxidative stress, extracellular matrix targets, and idiopathic ...
WEBSep 1, 2010 · Idiopathic pulmonary fibrosis (IPF) is an interstitial lung disease characterized by progressive fibrosis of the alveolar interstitium. The pathogenesis is thought to involve abnormal reepithelialization and dysregulated remodeling of the extracellular matrix after alveolar injury.
Idiopathic pulmonary fibrosis - The Lancet
WEBMay 13, 2017 · 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 destroyed, which leads to decreased lung compliance, disrupted gas exchange, and ultimately respiratory failure and death.
Idiopathic pulmonary fibrosis | Nature Reviews Disease Primers
WEBOct 20, 2017 · 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 is...
Extracellular matrix remodeling in idiopathic pulmonary fibrosis…
WEBCurrent disease pathogenesis assumes an aberrant wound healing process in response to repetitive injurious stimuli leading to apoptosis of epithelial cells, activation of fibroblasts and accumulation of extracellular matrix (ECM).
Complex Involvement of the Extracellular Matrix, Immune Effect, …
WEBJan 31, 2022 · Conclusion: Our data revealed a comprehensive transcriptional mRNA profile of IPF and demonstrated that ECM remodeling, lipid metabolism, and immune effect were collaboratively involved in the early development of IPF. Keywords: extracellular matrix remodeling; idiopathic pulmonary fibrosis; immune effect; lipid …