摘要：目的：探讨风湿性疾病继发肺动脉高压的影像学诊断及临床处理。方法：对30例本病住院患者的临床资料作回顾性研究。其中继发于系统性红斑狼疮17例,混合性结缔组织病8例,系统性硬化症5例。结果：患者的主要症状为胸闷、气急干咳,心脏彩超(UCG)均显示右室扩张伴三尖瓣返流。3年中死亡11例(36.8%)。重度肺动脉高压患者(肺动脉收缩压大于60mmHg)20例,死亡7例。早期诊断肺动脉高压6例,无1例死亡。静脉应用环磷酰胺(CTX) 冲击治疗患者的死亡率显著低于非用药组。26例患者短期应用前列地尔2周后,肺动脉收缩压平均降幅7.2%。结论：风湿性疾病继发肺动脉高压预后不良。UCG有助于早期发现肺动脉高压,CTX冲击治疗对改善预后有积极作用。早期诊断和治疗是降低死亡率的关键。

    关键词：风湿性疾病; 动脉高压; 心脏彩超

    The Diagnosis of Pulmonary Hypertension Secondary to Rheumatic by Ultrasonic Cardiogram

    WU Dai-hong, ZHANG Xiao-man

    (The First People's Hospital of Guangzhou,Guangdong Guangzhou 510180, China)

    Abstract： Objective： To analyze the imaging characteristics and management of pulmonary hypertension secondary to rheumatic diseases. Method： 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). Result： 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. ......