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目的探讨风湿性疾病继发肺动脉高压的临床特点及预后相关因素。 方法对 18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 11例 ,混合性结缔组织病 6例 ,系统性硬化症 1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 7例 (38.9% )。重度肺动脉高压患者 (肺动脉收缩压大于 6 0mmHg) 10例 ,死亡 5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。 7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。早期诊断和治疗是降低死亡率的关键。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。前列地尔的应用有待进一步探讨。
Objective To investigate the clinical features and prognostic factors of pulmonary hypertension secondary to rheumatic diseases. Methods The clinical data of 18 patients hospitalized for this disease were retrospectively studied. Which occurred in 11 cases of systemic lupus erythematosus, mixed connective tissue disease in 6 cases, 1 case of systemic sclerosis. Results The main symptoms of the patients were chest tightness, dry cough, and echocardiography (UCG) with right ventricular dilatation and tricuspid regurgitation. Seven died in three years (38.9%). 10 patients with severe pulmonary hypertension (pulmonary systolic blood pressure greater than 60mmHg) in 10 cases, 5 patients died. Early diagnosis of pulmonary hypertension in 6 cases, no one died. Mortality in patients treated with intravenous cyclophosphamide (CTX) was significantly lower than in non-medication groups. Short-term application of alprostadil in 7 patients resulted in an average 7.2% reduction in pulmonary systolic pressure after 2 weeks. Conclusions The prognosis of pulmonary hypertension secondary to rheumatic diseases is poor. Early diagnosis and treatment are the key to reducing mortality. UCG helps early detection of pulmonary hypertension, and CTX shock therapy has a positive effect on prognosis. The application of alprostadil remains to be further explored.