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]
© The Author(s). 2017. Licensed under Creative Commons Attribution 4.0 International (CC BY 4.0) http://creativecommons.org/licenses/by/4.0/
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 Type:||Refereed Article|
|Research Division:||Medical and Health Sciences|
|Research Group:||Clinical Sciences|
|Research Field:||Rheumatology and Arthritis|
|Objective Group:||Clinical Health (Organs, Diseases and Abnormal Conditions)|
|Objective Field:||Skeletal System and Disorders (incl. Arthritis)|
|Author:||Zochling, J (Dr Jane Zochling)|
|Web of Science® Times Cited:||7|
|Deposited By:||Menzies Institute for Medical Research|
|Downloads:||106 View Download Statistics|
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