ADAMTS13活性减低是抗磷脂抗体阳性患者血栓形成的危险因素

翻译：北医三院刘佩玲<[e-mail]>  

校正：于若寒     发布：熊丽桂

摘要：背景：抗磷脂抗体综合征（APS）是一种伴有抗磷脂抗体（aPLs）形成的自身免疫性疾病。先前的研究表明APS与血栓性血小板减少性紫癜可同时存在，因此我们假设抗ADAMTS13抗体也参与APS的病理生理过程。我们调查了aPLs阳性患者的ADAMTS13活性及抗ADAMTS13抗体的临床意义。

方法：研究纳入了216例狼疮抗凝物和（或）抗心磷脂抗体阳性的患者。分别使用荧光共振能量传递技术和ELISA测量ADAMTS13活性及抗ADAMTS13抗体。

结果：40.3%aPLs阳性患者（87/216）发现ADAMTS13活性减低。33.8%的患者（73/216）出现抗ADAMTS13抗体，其中41例患者的ADAMTS13活性正常。ADAMTS13活性减低是血栓形成的显著危险因素。血栓事件和年龄可导致ADAMTS13活性水平降低。抗ADAMTS13抗体的出现与ADAMTS13活性水平无关。自身免疫病患者往往有更高水平的ADAMTS13抗体。

结论：我们的研究发现ADAMTS13活性减低是aPLs阳性患者的血栓形成显著危险因素，而与抗ADAMTS13抗体出现无关。抗ADAMTS13抗体不导致ADAMTS13活性减低，而在自身免疫病患者中水平升高。

附原文：Antiphospholipid syndrome (APS) is an autoimmune disease in which antiphospholipid antibodies (aPLs) are generated. Previous studies show concurrence of APS and thrombotic thrombocytopenic purpura; therefore it is plausible to assume that anti-ADAMTS13 autoantibody is also involved in the pathophysiology of APS. We investigated the clinical significance of ADAMTS13 activity and anti-ADAMTS13 antibody in patients with aPLs. Two hundred and sixteen patients with positive lupus anticoagulant and/or anticardiolipin antibody were included. ADAMTS13 activity and anti-ADAMTS13 antibody were measured using fluorescence resonance energy-transfer technology and ELISA, respectively. Reduced ADAMTS13 activity was observed in 40.3% (87/216) of patients with aPLs. Although 33.8% (73/216) of patients were positive for anti-ADAMTS13 antibody, 41 of these 73 patients had normal levels of ADAMTS13 activity. Reduced ADAMTS13 activity was a significant risk factor for thrombotic events. Thrombotic events and age contributed to the reduced level of ADAMTS13 activity. Presence of anti-ADAMTS13 antibody did not show any association with the level of ADAMTS13 activity. Patients with autoimmune diseases tended to show higher levels of anti-ADAMTS13 antibody. Our findings suggest that reduced ADAMTS13 activity is a significant thrombotic risk factor in patients with aPLs irrespective of the presence of anti-ADAMTS13 antibody. Presence of anti-ADAMTS13 antibody is not seen with reduced activity and it tends to be increased in patients with autoimmune diseases.

引自：Lee SJ, Kim JE, Han KS, Kim HK.Thrombotic risk of reduced ADAMTS13 activity in patients with antiphospholipid antibodies.Blood Coagul Fibrinolysis. 2016,27(8):907-912.