Comparing prognosis in patients with pulmonary arterial hypertension

June 1, 2006

According to previous reports, patients with pulmonary arterial hypertension (PAH) associated with collagen-vascular disease have a better prognosis than patients who have idiopathic PAH. However, in a study conducted in Korea, Chung and colleagues recently found that the mortality rate was significantly higher in patients with systemic lupus erythematosus and pulmonary hypertension (SLE-PH) than in those with idiopathic PAH.

According to previous reports, patients with pulmonary arterial hypertension (PAH) associated with collagen-vascular disease have a better prognosis than patients who have idiopathic PAH. However, in a study conducted in Korea, Chung and colleagues recently found that the mortality rate was significantly higher in patients with systemic lupus erythematosus and pulmonary hypertension (SLE-PH) than in those with idiopathic PAH.

The authors compared the clinical features, laboratory and imaging findings at diagnosis, and survival rates in 34 patients with idiopathic PAH and 20 patients with SLE-PH. Tests performed included chest radiography, electrocardiography, echocardiography, pulmonary perfusion scans, and pulmonary function tests. The mean follow-up period was 18.1 months for the SLE-PH group and 33 months for the idiopathic PAH group.

During follow-up, the mortality rate was 60% in the SLE-PH group compared with 32% in the idiopathic PAH group. The 3-year survival rate was 44.9% in the SLE- PH group and 73.4% in the idiopathic PAH group. The respective 5-year survival rates were 16.8% and 68.2%.

The 2 groups did not differ significantly with respect to other clinical or laboratory findings.