Journal article

Screening for pulmonary arterial hypertension in systemic sclerosis: Now or never!

Zoe R Brown, Mandana Nikpour

EUROPEAN JOURNAL OF RHEUMATOLOGY | AVES | Published : 2020

Abstract

Systemic sclerosis (SSc), a chronic multisystem autoimmune disease characterized by fibrosis of the skin and internal organs and vasculopathy, has a high burden of mortality. One of the major contributors to mortality in patients with SSc is pulmonary arterial hypertension (PAH), which affects up to 10% of individuals and results in up to 15 years of life loss. Best practice recommendations are for asymptomatic patients with SSc and SSc-spectrum disorder to be screened annually for the early detection of SSc-PAH. Recently published data from large registries have shown improvements in the long-term outcomes in patients who are diagnosed with SSc-PAH because of systematic annual screening. Th..

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Grants

Funding Acknowledgements

Z.R.B. is supported by the research funding support from the Australian Commonwealth Government through the University of Melbourne Research Training Program Scholarship. M.N. is supported by grant from the Actelion.