Objective: The prognosis of systemic lupus erythematosus (SLE) patients with pulmonary hypertension (PH) was reported to be very poor and the median duration of survival estimated to be within 2 years. This study was designed to assess the clinical outcome and risk factors for poor prognosis of patients with SLE combined with PH. Methods: Two hundred ninety-two patients with SLE in whom echocardiography was performed were studied. The diagnosis of PH was made when the right ventricular systolic pressure (RVSP), measured by 2-dimensional echocardiography, was ＞30 mmHg. Demographic data, clinical manifestations, laboratory findings, and outcome of those with PH were evaluated. Results: Twenty-one of 292 patients had PH. The mean age at diagnosis of PH was 33.8⁑12.5 years. The most frequent presenting symptoms were dyspnea on exertion and dyspnea. Eight of 21 patients died after 1.3⁑1.1 years from diagnosis of PH. Survival rate at first and third year were 86% and 66%, respectively, and the median duration of survival estimated 3.0 years. SLE disease activity index (SLEDAI) and damage index at diagnosis of PH were significantly higher in the deceased. Among the patients in whom sequential echocardiography was performed, RVSP were significantly lowered in the survivors. High SLEDAI and high damage index were independent risk factors for poor prognosis. Conclusion: The survival rates and the median duration of survival were slightly better than those of previous reports. The high SLEDAI and high damage index were risk factors for poor prognosis of patients with SLE combined with PH.

Keywords: Systemic lupus erythematosus, Pulmonary hypertension, Clinical outcome, Survival rate