Pulmonary hypertension in systemic sclerosis

Pulmonary arterial hypertension occurs in up to 15% of patients who have systemic sclerosis (SSc) and has a high mortality. It can develop as an isolated complication or secondary to pulmonary fibrosis. There have been significant advances in assessment and therapy for this complication. Patients should be screened regularly by Doppler-echocardiography and pulmonary function tests. Right heart catheterization provides important diagnostic and prognostic information. Drawing from experience with treating primary pulmonary hypertension, treatment in the context of SSc is now possible. Patients should receive oral anticoagulation and oxygen supplementation. Calcium channel blockers are rarely effective, but parenteral prostacyclin analogs improve functional capacity and pulmonary hemodynamics. The oral endothelin-receptor blocker, bosentan, was shown to be an effective therapy for established symptomatic pulmonary hypertension in SSc. The next major challenge is to improve diagnosis and treatment of early stage or presymptomatic pulmonary hypertension with the goal of preventing this important cause of premature SSc-related mortality.