Bennett P. Deboisblanc, MD

abstract: Pulmonary arterial hypertension (PAH) is a common complication in patients with systemic sclerosis and is associated with an increased mortality rate. Patients are often asymptomatic early in the disease, but as the disease progresses, exertional dyspnea and fatigue develop. The workup usually includes chest radiography, pulmonary function tests, and Doppler echocardiography. If the results of Doppler echocardiography are consistent with PAH, the patient should undergo right heart catheterization. Patients with mild PAH who demonstrate considerable vasoreactivity are potential candidates for treatment with oral calcium channel blockers. Other therapies that can reduce symptoms and improve exercise tolerance and hemodynamics include bosentan and epoprostenol.