title: Baseline vWF factor predicts the development of elevated pulmonary artery pressure in systemic sclerosis.
abstract: OBJECTIVE
  This study aims to examine the utility of von Willebrand factor (vWF) as a biomarker in lcSSc, in particular the ability of vWF to predict the future development of disease manifestations in this disease.
  
  
  METHODS
  vWFAg concentrations were measured in the serum of patients with lcSSc at baseline and at 3 years, during the QUINs trial [Prevention of Vascular Damage in Scleroderma with Angiotensin-Converting Enzyme (ACE) Inhibition]. %DL(CO), %KCO, %FVC, pulmonary artery pressure (PAP) estimation by echocardiography, Raynaud's attack frequency, Raynaud's severity, digital ulcer frequency, urinary protein excretion, estimated glomerular filtration rate (eGFR), modified Rodnan skin score and Medsger disease activity score were also measured at baseline and 3 years.
  
  
  RESULTS
  Baseline serum vWF concentrations were related to concurrent Medsger disease activity score, %DL(CO), %FVC, urinary protein excretion, eGFR and PAP >30 mmHg. In logistic regression models, baseline serum vWF concentrations were able to predict the future development of elevated PAP by echocardiography (PAP >40 mmHg, P = 0.001).
  
  
  CONCLUSIONS
  Pulmonary artery hypertension is a life-threatening complication of lcSSc. vWF is a marker of endothelial cell activation. Raised serum concentrations of vWF in lcSSc increase the risk of developing subsequent elevation in PAP. Therefore screening patients with lcSSc for vWF may identify a group at risk of developing PAH. These patients could potentially be targeted with agents that stabilize the endothelium, e.g. statins.
authors.affiliation: Institute of Chronic Disease and Ageing, Clinical Sciences Centre, Aintree University Hospital, Longmoor Lane, Liverpool, UK.
authors.forename: Theresa
authors.initials: T
authors.lastname: Barnes
authors.forename: Angela
authors.initials: A
authors.lastname: Gliddon
authors.forename: Caroline J
authors.initials: CJ
authors.lastname: Doré
authors.forename: Peter
authors.initials: P
authors.lastname: Maddison
authors.forename: Robert J
authors.initials: RJ
authors.lastname: Moots
mesh_terms: D015415:Biomarkers / Q000097:blood*; D018450:Disease Progression; D004452:Echocardiography; D006801:Humans; D006976:Hypertension, Pulmonary / Q000097:blood* / Q000175:diagnosis / Q000209:etiology; D016015:Logistic Models; D008403:Mass Screening / Q000379:methods; D011237:Predictive Value of Tests; D011379:Prognosis; D011651:Pulmonary Artery / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology; D012595:Scleroderma, Systemic / Q000097:blood* / Q000150:complications / Q000175:diagnosis; D012720:Severity of Illness Index; D014841:von Willebrand Factor / Q000378:metabolism*
chemical_list: D015415:Biomarkers; D014841:von Willebrand Factor