Advances in Management of Pulmonary Hypertension Associated with Systemic Sclerosis

Abstract

Pulmonary hypertension is a well-known complication of systemic sclerosis. Patients with systemic sclerosis may develop a pulmonary arteriopathy characterized by vascular remodeling, increased pulmonary vascular resistance, and right ventricular failure. Pulmonary hypertension may also arise in systemic sclerosis as a consequence of interstitial lung disease or left ventricular dysfunction. Vascular remodeling is more prevalent than other forms of pulmonary hypertension in systemic sclerosis. The pathogenesis of pulmonary vascular remodeling in this disease state is not completely understood; however, there is evidence of a complex process involving genetic susceptibility, risk factors, vascular injury, and endothelial dysfunction. In those patients with pulmonary arterial hypertension, survival prognosis is extremely poor if the diagnosis is delayed or goes undetected and untreated. In recent years, a number of disease-targeted therapies have been developed that improve functional capacity, hemodynamics, and survival. Early detection and treatment with one or more targeted therapies are essential to improving survival when systemic sclerosis is complicated by pulmonary arterial hypertension.