Please use this identifier to cite or link to this item:
https://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):122-122 |
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. |
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 |
Published version: | http://dx.doi.org/10.1186/s13075-017-1341-x |
Appears in Collections: | Aurora harvest 8 Public Health publications |
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hdl_106529.pdf | Published Version | 1.35 MB | Adobe PDF | View/Open |
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