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Survival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertension


Morrisroe, K and Stevens, W and Huq, M and Prior, D and Sahhar, J and Ngian, G-S and Celermajer, D and Zochling, J and Proudman, S and Nikpour, M, and the Australian Scleroderma Interest Group (ASIG), Survival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertension, Arthritis Research & Therapy, 19, (1) Article 122. ISSN 1478-6354 (2017) [Refereed Article]


Copyright Statement

The Author(s). 2017. Licensed under Creative Commons Attribution 4.0 International (CC BY 4.0)

DOI: doi:10.1186/s13075-017-1341-x


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.

Cconclusions: 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.

Item Details

Item Type:Refereed Article
Research Division:Biomedical and Clinical Sciences
Research Group:Clinical sciences
Research Field:Rheumatology and arthritis
Objective Division:Health
Objective Group:Clinical health
Objective Field:Clinical health not elsewhere classified
UTAS Author:Zochling, J (Dr Jane Zochling)
ID Code:117413
Year Published:2017
Web of Science® Times Cited:40
Deposited By:Menzies Institute for Medical Research
Deposited On:2017-06-13
Last Modified:2018-06-04
Downloads:293 View Download Statistics

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