Vascular involvement in idiopathic pulmonary fibrosis.

ABSTRACT:

Background

Idiopathic pulmonary fibrosis (IPF) is a chronic, fibrosing and progressive interstitial lung disease of unknown aetiology with a pathogenesis still partly unknown. Several microvascular and macrovascular abnormalities have been demonstrated in the pathogenesis of IPF and related pulmonary hypertension (PH), a complication of the disease.

Methods

We carried out a non-systematic, narrative literature review aimed at describing the role of the vasculature in the natural history of IPF.

Results

The main molecular pathogenetic mechanisms involving vasculature (i.e. endothelial-to-mesenchymal transition, vascular remodelling, endothelial permeability, occult alveolar haemorrhage, vasoconstriction and hypoxia) and the genetic basis of vascular remodelling

SUBMITTER: Mondoni M 

PROVIDER: S-EPMC11587140 | biostudies-literature | 2024 Nov

REPOSITORIES: biostudies-literature