Publication at Faculty of Medicine in Hradec Králové |
2008
Abstract
Pulmonary arterial hypertension (PAH) is a serious complication of systemic sclerosis (SSc), having the most profound effect on prognosis, as pulmonary complications (PAH and pulmonary fibrosis) are the most common cause of death in SSc. However, progress continues to be made in the treatment of PAH, with data accumulating from subgroup analyse of several pivotal PAH studies, as well as from dedicated PAH-SSc studies and 'real practice'.
New therapies include the endothelin receptor antagonists, the phosphodiesterase-5 inhibitor and prostanoids.