Survival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertension
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 SS...
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doaj-d4c8d23868d749c08a685327102dcc3c2020-11-24T20:58:33ZengBMCArthritis Research & Therapy1478-63622017-06-0119111010.1186/s13075-017-1341-xSurvival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertensionKathleen Morrisroe0Wendy Stevens1Molla Huq2David Prior3Jo Sahhar4Gene-Siew Ngian5David Celermajer6Jane Zochling7Susanna Proudman8Mandana Nikpour9the Australian Scleroderma Interest Group (ASIG)Department of Medicine, The University of Melbourne at St Vincent’s HospitalDepartment of Rheumatology St Vincent’s HospitalDepartment of Medicine, The University of Melbourne at St Vincent’s HospitalDepartment of Medicine, The University of Melbourne at St Vincent’s HospitalMonash University and Monash HealthMonash University and Monash HealthThe University of Sydney at Royal Prince Alfred HospitalDepartment of Rheumatology, Menzies Institute for Medical ResearchRheumatology Unit, Royal Adelaide HospitalDepartment of Medicine, The University of Melbourne at St Vincent’s HospitalAbstract 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.http://link.springer.com/article/10.1186/s13075-017-1341-x |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Kathleen Morrisroe Wendy Stevens Molla Huq David Prior Jo Sahhar Gene-Siew Ngian David Celermajer Jane Zochling Susanna Proudman Mandana Nikpour the Australian Scleroderma Interest Group (ASIG) |
spellingShingle |
Kathleen Morrisroe Wendy Stevens Molla Huq David Prior Jo Sahhar Gene-Siew Ngian David Celermajer Jane Zochling Susanna Proudman Mandana Nikpour the Australian Scleroderma Interest Group (ASIG) Survival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertension Arthritis Research & Therapy |
author_facet |
Kathleen Morrisroe Wendy Stevens Molla Huq David Prior Jo Sahhar Gene-Siew Ngian David Celermajer Jane Zochling Susanna Proudman Mandana Nikpour the Australian Scleroderma Interest Group (ASIG) |
author_sort |
Kathleen Morrisroe |
title |
Survival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertension |
title_short |
Survival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertension |
title_full |
Survival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertension |
title_fullStr |
Survival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertension |
title_full_unstemmed |
Survival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertension |
title_sort |
survival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertension |
publisher |
BMC |
series |
Arthritis Research & Therapy |
issn |
1478-6362 |
publishDate |
2017-06-01 |
description |
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. |
url |
http://link.springer.com/article/10.1186/s13075-017-1341-x |
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