翻譯：北醫(yī)三院劉佩玲<276579712@qq.com>
校正：于若寒 發(fā)布：熊麗桂
摘要：背景：抗磷脂抗體綜合征（APS）是一種伴有抗磷脂抗體（aPLs）形成的自身免疫性疾病。先前的研究表明APS與血栓性血小板減少性紫癜可同時存在，因此我們假設抗ADAMTS13抗體也參與APS的病理生理過程。我們調(diào)查了aPLs陽性患者的ADAMTS13活性及抗ADAMTS13抗體的臨床意義。
方法：研究納入了216例狼瘡抗凝物和（或）抗心磷脂抗體陽性的患者。分別使用熒光共振能量傳遞技術(shù)和ELISA測量ADAMTS13活性及抗ADAMTS13抗體。
結(jié)果：40.3%aPLs陽性患者（87/216）發(fā)現(xiàn)ADAMTS13活性減低。33.8%的患者（73/216）出現(xiàn)抗ADAMTS13抗體，其中41例患者的ADAMTS13活性正常。ADAMTS13活性減低是血栓形成的顯著危險因素。血栓事件和年齡可導致ADAMTS13活性水平降低。抗ADAMTS13抗體的出現(xiàn)與ADAMTS13活性水平無關(guān)。自身免疫病患者往往有更高水平的ADAMTS13抗體。
結(jié)論：我們的研究發(fā)現(xiàn)ADAMTS13活性減低是aPLs陽性患者的血栓形成顯著危險因素，而與抗ADAMTS13抗體出現(xiàn)無關(guān)。抗ADAMTS13抗體不導致ADAMTS13活性減低，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.
