Update in systemic sclerosis-associated pulmonary arterial hypertension.

Abstract

Pulmonary arterial hypertension (PAH) is one of the leading causes of death in systemic sclerosis (SSc). Despite advances in treatment options for PAH, long-term prognosis remains poor for scleroderma-associated PAH (SSc-PAH). Although prompt diagnosis and treatment of PAH may have significant impact on survival rates, early detection of the syndrome continues to be challenging in SSc due to several factors ranging from limitations of the current screening tools and the complexities of the disease. In comparison with other PAH subgroups, SSc-PAH patients respond poorly to conventional forms of PAH therapy. Recent findings indicate that factors such as autoimmune and inflammatory responses, more severe vasculature remodeling, and intrinsic cardiac involvement may account for these differences.

• Authors: Mohamed A Gashouta, Marc Humbert, Paul M Hassoun
• Journal: Presse medicale (Paris, France : 1983) (Presse Med) Vol. 43 Issue 10 Pt 2 Pg. e293-304 (Oct 2014) ISSN: 2213-0276 [Electronic] France
• PMID: 25179278 (Publication Type: Journal Article, Review)
• Copyright: Copyright © 2014 Elsevier Masson SAS. All rights reserved.

Topics

• Humans
• Hypertension, Pulmonary (diagnosis, etiology, therapy)
• Scleroderma, Systemic (complications)