Systemic Sclerosis-Associated Pulmonary Arterial Hypertension

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...

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...

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...

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

The heart and pulmonary arterial hypertension in systemic sclerosis.

Systemic sclerosis (SSc) is an autoimmune connective tissue disease characterized by vasculopathy and progressive fibrosis of the skin and visceral organs (gastrointestinal tract, heart, kidneys and lungs). Although the prevalence is low, SSc is a disease with high morbidity and mortality. Since pulmonary arterial hypertension (PAH) associated with SSc (SSc-PAH) and clinically evident cardiac i...