Clinical Analysis of 30 Patients with Pulmonary Hypertension Secondary to Rheumatic Diseases
Objective To analyze the imaging characteristics and management of pulmonary hypertension secondary to rheumatic diseases.Methods A retrospective analysis was carried out in 30 patients with pulmonary hypertension (PHT) secondary to rheumatic diseases including 17 with systemic lupus erythematosus (SLE) , 8 with mixed connective tissue disease (MCTD) and 5 with systemic sclerosis (SSc).Results The main symptoms of the patients were dyspnea and non - productive cough. Ultrasonic cardiogram (UCG) revealed the dilated right ventricle with tricuspid regurgitation. 11 patients (36. 8%) died within three years. 7 of the 20 severe cases of pulmonary arterial systolic pressure (PASP) over 60mmHg died. 6 patients with early diagnosis all survived. Patients receiving the regular intravenous bolus therapy of cyclophosphamide (CTX) had better prognosis. A short course of intravenous infusion of alprostadil at a dose of 10μg daily for two weeks resulted in decreasing PAS by 7.2 %. Conclusion The prognosis of PHT secondary to rheumatic diseases is pessimistic. Early diagnosis and proper treatment are the keys to decrease the mortality rate. UCG is efficient for early diagnosis of PHT. CTX bolus therapy may improve the overall prognosis. Further evaluation of alprostadil therapy is warranted.