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Management of Pulmonary Arterial Hypertension in Patients with Systemic Sclerosis

Authors Almaaitah S, Highland KB, Tonelli AR

Received 22 September 2019

Accepted for publication 5 March 2020

Published 23 March 2020 Volume 2020:13 Pages 15—29


Checked for plagiarism Yes

Review by Single anonymous peer review

Peer reviewer comments 2

Editor who approved publication: Professor Turgay Celik

Saja Almaaitah,1 Kristin B Highland,2 Adriano R Tonelli2

1Medicine Institute, Cleveland Clinic, Cleveland, OH, USA; 2Department of Pulmonary and Critical Care Medicine, Cleveland Clinic, Cleveland, OH, USA

Correspondence: Adriano R Tonelli Email

Abstract: Systemic sclerosis (SSc) is a rare and complex immune-mediated connective tissue disease characterized by multi-organ fibrosis and dysfunction. Systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH) is a leading cause of death in this population. Pulmonary arterial hypertension (PAH) can coexist with other forms of pulmonary hypertension in SSc, including pulmonary hypertension related to left heart disease, interstitial lung disease, chronic thromboembolism and pulmonary venous occlusive disease, which further complicates diagnosis and management. Available pulmonary arterial hypertension therapies target the nitric oxide, endothelin and prostacyclin pathways. These therapies have been studied in SSc-PAH in addition to idiopathic PAH, often with different treatment responses. In this article, we discuss the management as well as the treatment options for patients with SSc-PAH.

Keywords: systemic sclerosis, scleroderma, pulmonary arterial hypertension, pulmonary hypertension

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