Pulmonary arterial hypertension associated with systemic sclerosis

Outcome measures in pulmonary arterial hypertension associated with systemic sclerosis.

SSc is complicated in approximately 10% of the patients by pulmonary arterial hypertension (PAH), a rare dyspnoea-fatigue syndrome caused by an increase in pulmonary vascular resistance. The prognosis of SSc-PAH is particularly poor, with estimated survival rates of approximately 50% at 2 yrs without pulmonary circulation-targeted therapies. Prostacyclins, endothelin receptor antagonists and ph...

Right ventricular dysfunction in systemic sclerosis-associated pulmonary arterial hypertension.

BACKGROUND Systemic sclerosis–associated pulmonary artery hypertension (SScPAH) has a worse prognosis compared with idiopathic pulmonary arterial hypertension (IPAH), with a median survival of 3 years after diagnosis often caused by right ventricular (RV) failure. We tested whether SScPAH or systemic sclerosis–related pulmonary hypertension with interstitial lung disease imposes a greater pulmo...

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 t...

Risk factors associated with pulmonary arterial hypertension in patients with systemic sclerosis and implications for screening.

Pulmonary arterial hypertension (PAH) is a relatively common complication of systemic sclerosis (SSc) affecting 5-12% of patients, and its development is associated with significant morbidity and a particularly poor prognosis. Deaths associated with other complications of SSc, such as renal crisis, have fallen significantly in recent years in line with improvements in their treatment and manage...

Different right ventricular contractility in limited cutaneous systemic sclerosis- associated pulmonary arterial hypertension and idiopathic pulmonary arterial hypertension