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


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Morrisroe, K, Stevens, W, Huq, M, Prior, D, Sahhar, J, Ngian, GS, Celermajer, D, Zochling, J, Proudman, S and Nikpour, M 2017 , 'Survival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertension' , Arthritis Research & Therapy, vol. 19, no. 1 , pp. 1-10 , doi: 10.1186/s13075-017-1341-x.

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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 p 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.

Item Type: Article
Authors/Creators:Morrisroe, K and Stevens, W and Huq, M and Prior, D and Sahhar, J and Ngian, GS and Celermajer, D and Zochling, J and Proudman, S and Nikpour, M
Journal or Publication Title: Arthritis Research & Therapy
Publisher: BioMed Central Ltd.
ISSN: 1478-6354
DOI / ID Number: 10.1186/s13075-017-1341-x
Copyright Information:

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

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