中文摘要：

目的：研究针对不同靶抗原的抗磷脂抗体与系统性红斑狼疮（SLE）合并肺动脉高压（PAH）的相关性。方法：收集72例SLE合并PAH（SLE-PAH）患者血清,固相酶联免疫吸附试验（ELISA）法检测血清抗心磷脂（ACL）抗体、抗β2糖蛋白Ⅰ抗体（抗β2-GPI）、抗凝血酶抗体、抗纤溶酶抗体、抗活化蛋白C抗体（抗APC）和抗组织型纤溶酶原激活物抗体（抗t-PA）,以128例肺动脉压力正常SLE（SLE-non-PAH）患者血清作为对照。结果：与SLE-non-PAH组相比,IgG型ACL抗体、抗β2-GPI抗体、抗纤溶酶抗体和抗凝血酶抗体阳性率在SLE-PAH组较高,两组间差异有统计学意义;抗APC抗体和抗t-PA抗体的检出率两组差异无统计学意义;重度SLE-PAH患者[肺动脉收缩压（PASP）≥60mmHg]的IgG型抗纤溶酶抗体阳性率较轻中度SLE-PAH患者（PASP〈60mmHg）高,两组间差异有统计学意义（P〈0.01）;SLE-PAH组和SLE-non-PAH组相比,各抗体IgM型差异无统计学意义。结论：IgG型抗心磷脂抗体、抗β2-GPI抗体、抗纤溶酶抗体和抗凝血酶抗体与SLE患者发生肺动脉高压相关。

英文摘要：

Objective To investigate the role of antiphospholipid antibodies against the target antigens in patients with systemic lupus erythematosus （SLE） associated with pulmonary arterial hypertension （PAH）. Methods Sera of 72 SLE patients with PAH （SLE-PAH） and sera of 128 SLE patients without PAH （non- PAH-SLE） were tested for antibodies to cardiolipin（CL）, β2-glycoprotein Ⅰ （β2-GPI）, plasmin, thrombin, activated protein C （APC） and tissue plasminogen activator （t-PA） by enzyme linked immunosorbent assay （ELISA）. Results The positive rate of IgG class of ACL, anti-β2-GPI anti-thrombin and anti-plasmin antibodies was higher in patients with SLE-PH than in non-PAH-SLE. The positive rates of antibodies to APC and t-PA were not statistically different between the SLE-PAH and non-PAH-SLE group. The positive rate of IgG anti-plasmin antibody in severe SLE-PH （PASP≥60 mm Hg） group was higher than that in mild to moderate SLE-PH （PASP〈60 mm Hg） groups （P=0.003）. No differences were found in antibody of IgM class between SLE-PAH and SLE-non-PAH groups. Conclusion Antiphospholipid antibodies to cardiolipin, β2-GPI, thrombin and plasmin play a role in the pathogenesis of SLE related pulmonary arterial hypertension.