title: Plasma endothelin-1 and vascular endothelial growth factor levels and their relationship to hemodynamics in idiopathic pulmonary fibrosis.
abstract: BACKGROUND
  Pulmonary hypertension (PH) is associated with a poor prognosis in idiopathic pulmonary fibrosis (IPF). Endothelin-1 (ET-1) and vascular endothelial growth factor (VEGF) are important in both fibrosis and vascular remodeling.
  
  
  OBJECTIVE
  We sought to determine the relationship between ET-1 and VEGF levels and hemodynamics in patients with IPF. We hypothesized that higher levels of ET-1 and VEGF would be associated with higher pulmonary artery pressures (PAP) and pulmonary vascular resistance (PVR) in patients with IPF.
  
  
  METHODS
  We performed a cross-sectional analysis of 52 adults with IPF enrolled in a prospective cohort with available clinical data, platelet-free plasma, and hemodynamics. ET-1 and VEGF levels were measured via immunoassay. The associations of ET-1 and VEGF with PAP and PVR were examined using generalized additive models adjusted for age, gender, race/ethnicity, and forced vital capacity (% predicted).
  
  
  RESULTS
  Sixteen of 52 (30.8%) had PH (mean PAP ≥25 mm Hg). After multivariable adjustment, higher ET-1 levels were significantly associated with higher systolic (p = 0.01), diastolic (p = 0.02), and mean (p = 0.01) PAP and possibly higher PVR (p = 0.09). There were no significant associations between VEGF levels and hemodynamics.
  
  
  CONCLUSIONS
  Higher levels of ET-1 were associated with higher PAP and possibly higher PVR in participants with IPF. In a subgroup of patients, ET-1 may be a contributor to pulmonary vascular disease burden in IPF.
authors.affiliation: Department of Medicine, Alpert Medical School of Brown University, Providence, RI, USA.
authors.forename: Corey E
authors.initials: CE
authors.lastname: Ventetuolo
authors.forename: Steven M
authors.initials: SM
authors.lastname: Kawut
authors.forename: David J
authors.initials: DJ
authors.lastname: Lederer
mesh_terms: D000368:Aged; D062186:Arterial Pressure; D003430:Cross-Sectional Studies; D019332:Endothelin-1 / Q000378:metabolism*; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology / Q000378:metabolism*; D054990:Idiopathic Pulmonary Fibrosis / Q000150:complications / Q000378:metabolism*; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D011651:Pulmonary Artery / Q000503:physiopathology*; D042461:Vascular Endothelial Growth Factor A / Q000378:metabolism*; D014655:Vascular Resistance
chemical_list: D019332:Endothelin-1; C467484:VEGFA protein, human; D042461:Vascular Endothelial Growth Factor A