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Therapeutic Challenges And Advances In The Management Of Systemic Sclerosis-Related Pulmonary Arterial Hypertension (SSc-PAH)

Authors Argula RG, Ward C, Feghali-Bostwick C

Received 15 August 2019

Accepted for publication 6 October 2019

Published 13 December 2019 Volume 2019:15 Pages 1427—1442


Checked for plagiarism Yes

Review by Single anonymous peer review

Peer reviewer comments 2

Editor who approved publication: Professor Garry Walsh

Rahul G Argula,1 Celine Ward,2 Carol Feghali-Bostwick2

1Department of Medicine, Division of Pulmonary and Critical Care Medicine, Medical University of South Carolina, Charleston, SC 29425, USA; 2Department of Medicine, Division of Rheumatology, Medical University of South Carolina, Charleston, SC 29425, USA

Correspondence: Rahul G Argula
Department of Medicine, Division of Pulmonary and Critical Care Medicine Medical University of South Carolina, 816 CSB, MSC 630, Jonathan Lucas Street, Charleston, SC 29425, USA
Tel +1 843 792 3167

Abstract: Systemic sclerosis (SSc) is a rare autoimmune disorder with multi-organ involvement. SSc-associated pulmonary arterial hypertension (SSc-PAH) is one of the leading causes of morbidity and mortality in the SSc population. With advances in our understanding of pulmonary arterial hypertension (PAH) diagnosis and treatment, outcomes for all PAH patients have significantly improved. While SSc-PAH patients have also benefited from these advances, significant challenges remain. Diagnosis of PAH is a challenging endeavor in SSc patients who often have many co-existing pulmonary and cardiac comorbidities. Given the significantly elevated prevalence and lifetime risk of PAH in the SSc population, screening for SSc-PAH is a critically useful strategy. Treatment with pulmonary arterial (PA) vasodilators has resulted in a dramatic improvement in the survival and quality of life of PAH patients. While therapy with PA vasodilators is beneficial in SSc-PAH patients, therapy effects appear to be attenuated when compared to responses in patients with idiopathic PAH (IPAH). This review attempts to chronicle and summarize the advances in our understanding of the optimal screening strategies to identify PAH in patients with SSc. The article also reviews the advances in the therapeutic and risk stratification strategies for SSc-PAH patients.

Keywords: systemic sclerosis, pulmonary arterial hypertension, screening, risk stratification, therapy, advances

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