Presentation: Predicting Pulmonary Artery Hypertension in Patients with Systemic Sclerosis. (2007)

19 Predicting Pulmonary Artery Hypertension in Patients with Systemic Sclerosis.

Purpose
Pulmonary artery hypertension (PAH) is a major cause of morbidity and mortality in patients with systemic sclerosis (SSc) and tends to be asymptomatic until advanced stages. New therapeutic strategies have led to improved symptoms and survival. The assumption, as yet unproven, is that the early detection of PAH may lead to improved outcomes by facilitating early intervention. This study aims to explore the utility of von Willebrand factor (vWF) as a biomarker to predict the development of PAH in SSc patients.
Methods
133 patients with SSc or Raynaud's phenomenon (with positive autoantibodies associated with SSc) had vWF measured at baseline and 3years as part of a large prospective study to evaluate the potential role of quinapril in reducing digital infarcts in the conditions. Patients also had an estimation of their pulmonary artery pressure, measured by echocardiography, at baseline and after 3years in the study programme.
Results
39 patients had a pulmonary artery pressure >30mmHg at 3years and 12 had a pulmonary artery pressure >40mmHg at 3years. Analysis by logistic regression using robust Huber-White standard errors of the regression coefficients to take account of the observed correlations between repeated assessments on the same patient revealed that elevated vWF (>160% NPP) predicted the development of pulmonary artery hypertension. Pulmonary artery pressure >30mmHg (p=0.03, odds ratio 3.2, 95% CI 1.2, 8.9), pulmonary artery pressure >40mmHg (p=0.02, odds ratio 11.1, 95% CI 1.5, 84).
Conclusion
In a large prospective study of patients with SSc or autoimmune Raynaud's, vWF >160% NPP appears to be a significant predictor for the development of PAH. Whilst the ability of vWF alone to act as a predictor of future PAH is imprecise, it is possible that this might be enhanced if used in combination with other biomarkers.

 T.C. Barnes, None; C. Dore, None; A. Gliddon, None; P. Maddison, None; R. Moots, None.