Screening and therapy of pulmonary hypertension in systemic sclerosis

Abstract

Purpose of review: Systemic sclerosis is a major risk factor for the development of pulmonary arterial hypertension. Often this disease is far advanced by the time it is detected in the individual patient, severely impacting quality of life and survival.

Recent findings: Therapeutic options available for patients with scleroderma-associated pulmonary arterial hypertension have increased significantly over the last few years, fueling our desire to improve early detection of disease.

Summary: During the course of this manuscript I will review the literature examining approaches towards screening for scleroderma-associated pulmonary arterial hypertension. I will then discuss the most recent studies examining therapeutic options for patients with this disease.