Journal article

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

K Morrisroe, W Stevens, M Huq, D Prior, J Sahhar, GS Ngian, D Celermajer, J Zochling, S Proudman, M Nikpour, C Hill, S Lester, P Nash, G Ngian, M Rischmueller, J Roddy, G Strickland, V Thakkar, J Walker

Arthritis Research and Therapy | BMC | Published : 2017

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 patient..

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