Please use this identifier to cite or link to this item: https://hdl.handle.net/2440/77980
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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.date.issued2012-
dc.identifier.citationArthritis Research and Therapy, 2012; 14(R213):1-9-
dc.identifier.issn1478-6354-
dc.identifier.issn1478-6362-
dc.identifier.urihttp://hdl.handle.net/2440/77980-
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.language.isoen-
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 (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.-
dc.source.urihttp://dx.doi.org/10.1186/ar4051-
dc.subjectConnective tissue disease-
dc.subjectmortality-
dc.subjectprognosis-
dc.titlePredictors of mortality in connective tissue disease-associated pulmonary arterial hypertension: a cohort study-
dc.typeJournal article-
dc.identifier.doi10.1186/ar4051-
pubs.publication-statusPublished-
dc.identifier.orcidHill, C. [0000-0001-8289-4922]-
dc.identifier.orcidProudman, S. [0000-0002-3046-9884]-
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