Pulmonary arterial hypertension in systemic sclerosis

Pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc) is a complex clinical situation resulting from restricted flow through the pulmonary arterial circulation ending in increased pulmonary vascular resistance and right heart failure. PAH is a common and life-threatening complication in connective tissue diseases, specifically in SSc if not treated rapidly and adequately. Based on the emerging knowledge in SSc epidemiology by large scale patient cohorts such as EUSTAR, of PAH pathophysiology and advances in cardiopulmonary diagnostic techniques, several novel treatment approaches have been examined and have proceeded to licensing and daily use in the clinical practice. Amongst them are different endothelin receptor antagonists and PDE-5 inhibitors, but several other ideas are being currently pursued to improve the long-term outcome of the affected patients.