Current therapies for the treatment of systemic sclerosis-related pulmonary arterial hypertension: efficacy and safety

Systemic sclerosis (SSc) is a rare connective tissue disease characterized by chronic inflammation and fibrosis of the skin, vascular abnormalities and variable involvement of organs. Patients with limited SSc typically develop pulmonary arterial hypertension (PAH). TNF-α, VEGF, platelet-derived growth factor and endothelin-1 play a key role in the development of PAH.This paper addresses the efficacy and safety of current drugs used for the...