Abstract
Idiopathic pulmonary fibrosis (IPF) is a fibrosing interstitial lung disease of unknown etiology. Different types of cells are involved in fibrogenesis, which is persistently physical and molecular stimulation, either directly or by interacting with bioactive molecules and extracellular vesicles (EVs). Current evidence suggests that EVs play an essential role in IPF development. EVs are released by a variety of cells, including fibroblasts, epithelial cells, and alveolar macrophages. In addition, EVs can transport bioactive molecules, such as lipids, proteins, and nucleic acids, which play a pivotal role in cellular communication. Several proposed mechanisms show that an acceptor cell can capture, absorb, or interact with EVs through direct fusion with the plasma membrane, ligand–receptor interaction, and endocytotic process, modifying the target cell. During fibrogenesis, the release of EVs is deregulated, increases the EVs amount, and the cargo content is modified. This alteration is closely associated with the maintenance of the fibrotic microenvironment. This review summarizes the current data on the participation of EVs secreted by the cells playing a critical role in IPF pathogenesis.
Highlights
Idiopathic pulmonary fibrosis (IPF) is fibrosing interstitial pneumonia of unknown etiology. It is a chronic and progressive disease characterized by aberrant deposition of extracellular matrix (ECM) in the lung parenchyma that deforms the alveolar architecture, causing respiratory failure as it decreases lung function and gas exchange, which can eventually cause death [1]
Previous studies have proposed that mesenchymal stem cells (MSCs) are responsible for maintaining vascular homeostasis and facilitating repair; contrarily, studies performed in lung tissue have reported that MSCs participates in the restoration of injured endothelium through the release of paracrine mediators, such as extracellular vesicles (EVs) and activation of different signaling pathways, including (IL-6), interleukin 6 receptor subunit alpha (IL-6RA), Janus kinase (JAK), signal transducer and activator of transcription 3 (STAT3) [61,62]
IPF is a chronic progressive pathology of unknown etiology, poor prognosis, and high mortality rate. This disease involves a heterogeneous group of cells such as AEC-I, AEC-II, macrophages, and fibroblasts that orchestrate a pathological state mediated by a microenvironment enriched with soluble factors associated with fibrosis development, such as cytokines, chemokines, and EVs production
Summary
Idiopathic pulmonary fibrosis (IPF) is fibrosing interstitial pneumonia of unknown etiology. It is a chronic and progressive disease characterized by aberrant deposition of extracellular matrix (ECM) in the lung parenchyma that deforms the alveolar architecture, causing respiratory failure as it decreases lung function and gas exchange, which can eventually cause death [1]. The synergistic effect of both internal and external factors are required for the initiation and progression of IPF mainly, in subjects with a genetic predisposition. Tissue alterations are established through recurrent lesions in the pulmonary epithelium superimposed on the accelerated aging of the epithelium itself, leading to an aberrant repair of lung parenchyma and causing excessive accumulation of ECM and all clinical features of the disease [1,2]
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