Survival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertension
AuthorMorrisroe, K; Stevens, W; Huq, M; Prior, D; Sahhar, J; Ngian, G-S; Celermajer, D; Zochling, J; Proudman, S; Nikpour, M
Source TitleARTHRITIS RESEARCH & THERAPY
University of Melbourne Author/sNgian, Gene-Siew; Huq, Molla; Nikpour, Mandana; Morrisroe, Kathleen Bridget; Prior, David; Stevens, Wendy
AffiliationMedicine, Dentistry & Health Sciences
Medicine (St Vincent's)
Document TypeJournal Article
CitationsMorrisroe, K., Stevens, W., Huq, M., Prior, D., Sahhar, J., Ngian, G. -S., Celermajer, D., Zochling, J., Proudman, S. & Nikpour, M. (2017). Survival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertension. ARTHRITIS RESEARCH & THERAPY, 19 (1), https://doi.org/10.1186/s13075-017-1341-x.
Access StatusOpen Access
Open Access at PMChttp://www.ncbi.nlm.nih.gov/pmc/articles/PMC5457656
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.
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