系统性红斑狼疮并发肺动脉高压16例临床分析

Clinical analysis of 16 cases of systemic lupus erythematosus complicated with pulmonary hypertension

目的：采用多普勒超声心动图对系统性红斑狼疮（SLE）患者进行肺动脉高压的筛查，并与疾病其他临床表现及实验室检查进行相关性分析。方法：138例SLE患者行多普勒超声检查，其中成人SLE患者132例，未成年人6例。收缩期峰值压力梯度在三尖瓣超过30mm Hg（1mmHg=0．133kPa）则诊断为肺动脉高压。所有患者均有完整的病史及实验室检查。结果：138例SLE患者中，16例（包括15例成人患者和1例未成年人患者）并发肺动脉高压（11．59％）。肺动脉收缩压范围42～85mmHg。并发肺动脉高压患者和无肺动脉高压患者的雷诺征及血管炎皮疹差异有统计学意义。而在实验室检查中，与无肺动脉高压患者相比，并发肺动脉高压患者出现类风湿因子和抗心磷脂抗体的频率明显升高。而补体C3、抗核抗体（ANA）及抗双链DNA（ds-DNA）抗体与肺动脉高压的发生无显著相关性。结论：雷诺征、血管炎皮疹、类风湿因子和抗心磷脂抗体阳性可作为SLE并发肺动脉高压的预测危险因素，超声心动图在SLE患者中可作为筛查肺动脉高压的一种有效的手段。

Objective： Doppler echocardiography was used to screen the pulmonary hypertension for the patients with systemic lupus erythematosus （SLE）, correlation analysis was performed between pulmonary hypertension and other clinical manifestations and laboratory examinations of the disease. Methods： Doppler echocardiography was performed in 138 patients with SLE（132 adult and 6 juvenile）. Pulmonary hypertension was diagnosed, if the peak systolic pressure gradient at the tricuspid valve was 〉 30mmHg. All patients had a complete medical history and laboratory examination. Results： In 138 SLE patients, 16 patients（including 15 adults and 1 juvenile） complicated with pulmonary hypertension（11.59%）. The range of systolic pressure of pulmonary artery was 42 to 85 mmHg. The difference of Raynaud syndrome and vasculitis lesion between the SLE patients complicated with pulmonary hypertension and the patients without pulmonary hypertension was statistically significant. Laboratory examination revealed the frequency of rheumatoid factor and anticardiolipin antibody significantly increased in the patients with pulmonary hypertension, compared with the patients without pulmonary hypertension. No significant correlation was found between pulmonary hypertension and complement C3, ANA and anti-dsDNA antibody. Conclusion： Raynaud syndrome, vasculitis rash, positive rheumatoid factor and antieardiolipin antibody can be used to predict the risk of the patients with SLE complicated with pulmonary hypertension, and echocardiography is an effective way of screening for puhnonary hypertension in SLE patients.