pulmonary arterial hypertension

Systemic sclerosis is a rare multisystem connective tissue disease characterized by a vasculopathy, diffuse fibrosis and immune abnormalities. This disease has a high mortality rate, especially if it presents with concomitant disorders, such as interstitial lung disease or pulmonary arterial hypertension. Although pulmonary arterial hypertension is a rare disease, with an estimated frequency of 15-50 cases per million, the prevalence of this condition in patients with systemic sclerosis has been reported to be 7-12%.