Disease progression in systemic sclerosis associated pulmonary arterial hypertension

Abstract

Systemic sclerosis associated pulmonary arterial hypertension (SSc-PAH) is an important cause of morbidity and mortality in patients with systemic sclerosis (SSc). Although studies based on echocardiography-derived pulmonary artery pressures have resulted in estimates of the prevalence of pulmonary arterial hypertension (PAH) in patients with SSc as high as 35%, more recent studies, using catheter-based diagnoses, have produced estimates of between 7.8 and 12%.1-3 Historically, SSc-PAH has had a poor outlook with rapid clinical deterioration and a 3-year survival of 30%.4 This was worse than in idiopathic pulmonary arterial hypertension (iPAH) wherein median survival prior to disease-modifying therapy was 2.8 years.5 © 2010 Springer-Verlag London.