Management of Pulmonary Arterial Hypertension in Patients with Systemic Sclerosis

Abstract

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, USACorrespondence: Adriano R Tonelli Email [email protected]: 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  

Keywords

• systemic sclerosis
• scleroderma
• pulmonary arterial hypertension
• pulmonary hypertension