Please use this identifier to cite or link to this item: http://hdl.handle.net/2440/106529
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Type: Journal article
Title: Survival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertension
Author: Morrisroe, K.
Stevens, W.
Huq, M.
Prior, D.
Sahhar, J.
Ngian, G.
Celermajer, D.
Zochling, J.
Proudman, S.
Nikpour, M.
Hill, C.
Lester, S.
Nash, P.
Ngian, G.
Rischmueller, M.
Roddy, J.
Sahhar, J.
Strickland, G.
Thakkar, V.
Walker, J.
Citation: Arthritis Research and Therapy, 2017; 19(1):122
Publisher: BioMed Central
Issue Date: 2017
ISSN: 1478-6354
1478-6362
Statement of
Responsibility: 
Kathleen Morrisroe, Wendy Stevens, Molla Huq, David Prior, Jo Sahhar, Gene-Siew Ngian, David Celermajer, Jane Zochling, Susanna Proudman, Mandana NikpourEmail author and the Australian Scleroderma Interest Group (ASIG)
Abstract: Background: Pulmonary arterial hypertension (PAH) is a leading cause of mortality in systemic sclerosis (SSc). We sought to determine survival, predictors of mortality, and health-related quality of life (HRQoL) related to PAH in a large SSc cohort with PAH. Methods: We studied consecutive SSc patients with newly diagnosed (incident) World Health Organization (WHO) Group 1 PAH enrolled in a prospective cohort between 2009 and 2015. Survival methods were used to determine age and sex-adjusted standardised mortality ratio (SMR) and years of life lost (YLL), and to identify predictors of mortality. HRQoL was measured using the Short form 36 (SF-36) instrument. Results: Among 132 SSc-PAH patients (112 female (85%); mean age 62 ± 11 years), 60 (45.5%) died, with a median (±IQR) survival time from PAH diagnosis of 4.0 (2.2-6.2) years. Median (±IQR) follow up from study enrolment was 3.8 (1.6-5.8) years. The SMR for patients with SSc-PAH was 5.8 (95% CI 4.3-7.8), with YLL of 15.2 years (95% CI 12.3-18.1). Combination PAH therapy had a survival advantage (p < 0.001) compared with monotherapy, as did anticoagulation compared with no anticoagulation (p < 0.003). Furthermore, combination PAH therapy together with anticoagulation had a survival benefit compared with monotherapy with or without anticoagulation and combination therapy without anticoagulation (hazard ratio 0.28, 95% CI 0.1-0.7). Older age at PAH diagnosis (p = 0.03), mild co-existent interstitial lung disease (ILD) (p = 0.01), worse WHO functional class (p = 0.03) and higher mean pulmonary arterial pressure at PAH diagnosis (p = 0.001), and digital ulcers (p = 0.01) were independent predictors of mortality. Conclusions: Despite the significant benefits conferred by advanced PAH therapies suggested in this study, the median survival in SSc PAH remains short at only 4 years.
Keywords: Australian Scleroderma Interest Group (ASIG); Humans; Hypertension, Pulmonary; Scleroderma, Systemic; Incidence; Quality of Life; Aged; Middle Aged; Female; Male
Rights: © The Author(s). 2017 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
RMID: 0030071194
DOI: 10.1186/s13075-017-1341-x
Grant ID: http://purl.org/au-research/grants/nhmrc/1113954
http://purl.org/au-research/grants/nhmrc/1071735
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