Journal article
Multicentre randomised placebo-controlled trial of oral anticoagulation with apixaban in systemic sclerosis-related pulmonary arterial hypertension: The SPHInX study protocol
A Calderone, W Stevens, D Prior, H Nandurkar, E Gabbay, SM Proudman, T Williams, D Celermajer, J Sahhar, PKK Wong, V Thakkar, N Dwyer, J Wrobel, W Chin, D Liew, M Staples, R Buchbinder, M Nikpour
BMJ Open | BMJ PUBLISHING GROUP | Published : 2016
Abstract
Introduction Systemic sclerosis (SSc) is a severe and costly multiorgan autoimmune connective tissue disease characterised by vasculopathy and fibrosis. One of the major causes of SSc-related death is pulmonary arterial hypertension (PAH), which develops in 12-15% of patients with SSc and accounts for 30-40% of deaths. In situ thrombosis in the small calibre peripheral pulmonary vessels resulting from endothelial dysfunction and an imbalance of anticoagulant and prothrombotic mediators has been implicated in the complex pathophysiology of SSc-related PAH (SSc-PAH), with international clinical guidelines recommending the use of anticoagulants for some types of PAH, such as idiopathic PAH. How..
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Awarded by National Science Foundation
Funding Acknowledgements
This study is financially supported by a peer-reviewed 5-year Project Grant (APP1062638) from the National Health and Medical Research Council of Australia (NHMRC). MN holds an NHMRC Research Fellowship (APP1071735). RB holds an NHMRC Senior Research Fellowship (APP1082138). The study drug and matching placebo are being supplied at no cost by Bristol-Myers Squibb Pty. Bristol-Myers Squibb were permitted to review the manuscript and make suggestions, but the final decision on content was exclusively retained by the authors. MN has received research support from Actelion, GlaxoSmithKline and Pfizer.