Abstract
BackgroundIdiopathic pulmonary fibrosis (IPF) has been associated with abnormal vascular remodeling. Bone marrow derived endothelial progenitor cells (EPCs) are considered to possess lung tissue repair and vascular remodeling properties.ObjectivesThe study aimed to assess early EPCs levels and EPCs endogenous vascular endothelial growth factor (VEGF) expression in IPF. In order to examine alterations in the mobilization of EPCs from the bone marrow we measured plasma VEGF.Main ResultsTwenty-three patients with IPF and fifteen healthy subjects were included. The number of early EPCs colonies was markedly reduced in IPF patients vs controls (6.00±6.49 vs 49.68±16.73, respectively, p<0.001). EPCs were further decreased in patients presenting systolic pulmonary arterial pressure (sPAP)≥35 mmHg. The number of colonies per well correlated negatively with P(A-a)O2 (r = −0.750, p<0.001). Additionally, VEGF mRNA levels were significantly increased in IPF patients. There were no differences observed in VEGF plasma levels in IPF patients when compared to controls.ConclusionsThe current data suggest that inadequate levels of early EPCs may potentially contribute to suppressed repair and recovery of the damaged pulmonary endothelium and thereby may drive the sequence of events in profibrogenic direction. Increased VEGFmRNA levels in the clinical context of IPF may represent a compensatory mechanism to overcome reduced EPCs levels.
Highlights
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrotic lung disease which is associated with lack of effective treatment and poor survival [1,2]
The number of early endothelial progenitor cells (EPCs) colonies was markedly reduced in IPF patients vs controls (6.0066.49 vs 49.68616.73, respectively, p,0.001)
There were no differences observed in vascular endothelial growth factor (VEGF) plasma levels in IPF patients when compared to controls
Summary
Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrotic lung disease which is associated with lack of effective treatment and poor survival [1,2]. Multiple mechanisms have been proposed to play a role in IPF pathogenesis including abnormal vascular repair and remodeling. There is a body of evidence suggesting that in IPF, the impairment of repair reendothelization mechanisms following alveolar injury may lead to destruction in lung architecture and fibrosis [3]. Reduced EPCs numbers have been associated with persistent fibrotic changes, not due to IPF, in humans following lung injury [5]. In this respect, one might argue that EPCs might play a pivotal role in IPF [8]. Bone marrow derived endothelial progenitor cells (EPCs) are considered to possess lung tissue repair and vascular remodeling properties
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