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dc.contributor.authorNgian, G.-
dc.contributor.authorStevens, W.-
dc.contributor.authorPrior, D.-
dc.contributor.authorGabbay, E.-
dc.contributor.authorRoddy, J.-
dc.contributor.authorTran, A.-
dc.contributor.authorMinson, R.-
dc.contributor.authorHill, C.-
dc.contributor.authorChow, K.-
dc.contributor.authorSahhar, J.-
dc.contributor.authorProudman, S.-
dc.contributor.authorNikpour, M.-
dc.identifier.citationArthritis Research and Therapy, 2012; 14(R213):1-9-
dc.descriptionExtent: 9p.-
dc.description.abstractIntroduction: Pulmonary arterial hypertension (PAH) is a major cause of mortality in connective tissue disease (CTD). We sought to quantify survival and determine factors predictive of mortality in a cohort of patients with CTD-associated PAH (CTD-PAH) in the current era of advanced PAH therapy. Methods: Patients with right heart catheter proven CTD-PAH were recruited from six specialised PAH treatment centres across Australia and followed prospectively. Using survival methods including Cox proportional hazards regression, we modelled for all-cause mortality. Independent variables included demographic, clinical and hemodynamic data. Results: Among 117 patients (104 (94.9%) with systemic sclerosis), during 2.6 ± 1.8 (mean ± SD) years of follow-up from PAH diagnosis, there were 32 (27.4%) deaths. One-, two- and three-year survivals were 94%, 89% and 73%, respectively. In multiple regression analysis, higher mean right atrial pressure (mRAP) at diagnosis (hazard ratio (HR) = 1.13, 95% CI: 1.04 to 1.24, P = 0.007), lower baseline six-minute walk distance (HR = 0.64, 95% CI: 0.43 to 0.97, P = 0.04), higher baseline World Health Organization functional class (HR = 3.42, 95% CI: 1.25 to 9.36, P = 0.04) and presence of a pericardial effusion (HR = 3.39, 95% CI: 1.07 to 10.68, P = 0.04) were predictive of mortality. Warfarin (HR = 0.20, 95% CI: 0.05 to 0.78, P = 0.02) and combination PAH therapy (HR = 0.20, 95% CI: 0.05 to 0.83, P = 0.03) were protective. Conclusions: In this cohort of CTD-PAH patients, three-year survival was 73%. Independent therapeutic predictors of survival included warfarin and combination PAH therapy. Our findings suggest that anticoagulation and combination PAH therapy may improve survival in CTD-PAH. This observation merits further evaluation in randomised controlled trials.-
dc.description.statementofresponsibilityGene-Siew Ngian, Wendy Stevens, David Prior, Eli Gabbay, Janet Roddy, Ai Tran, Robert Minson, Catherine Hill, Ken Chow, Joanne Sahhar, Susanna Proudman and Mandana Nikpour-
dc.publisherBioMed Central Ltd.-
dc.rights© 2012 Ngian et al.; licensee BioMed Central Ltd.This is an open access article distributed under the terms of the Creative Commons Attribution License (, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.-
dc.subjectConnective tissue disease-
dc.titlePredictors of mortality in connective tissue disease-associated pulmonary arterial hypertension: a cohort study-
dc.typeJournal article-
dc.identifier.orcidHill, C. [0000-0001-8289-4922]-
dc.identifier.orcidProudman, S. [0000-0002-3046-9884]-
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