血管性血友病因子裂解蛋白酶活性在系统性红斑狼疮并抗磷脂综合征中的临床意义

Clinical significance of von Willebrand factor cleaving protease activity in patients with systemic lupus erythematosus and antiphospholipid syndrome

目的了解血管性血友病因子裂解蛋白酶(ADAMT-13)活性与系统性红斑狼狼疮(SLE)合并抗磷脂综合征(APS)临床特点的关系以及其价值。方法采用残余胶原结合试验检测22例SLE患者、13例SLE并APS(SLE-APS)患者和22例正常人血清中的ADAMT-13水平,并分析其与SLE-APS患者及SLE患者临床表现、实验室指标、疾病活动度及预后的关系。统计学分析采用t检验、χ~2检验和Spearman检验。结果 (1)SLE-APS患者的血清ADAMTS-13活性下降,且与血栓及血小板减少的表现相关。(2)SLE-APS患者的血清ADAMTS-13活性与SLE疾病活动度成反比。结论 SLE-APS的患者可能存在更多的自身抗体以及存在更严重的血栓表现及血小板减少,因此可能导致体内血管性血友病因子(vWF)生成增加;存在抗ADAMTS-13抗体,从而最终导致血清ADAMTS-13的活性下降。

Objective To investigate the von Willebrand factor cleaving protease(a disintegrin and metalloprotease with a thrombo Spondin type 1 motif-member 13, ADAMT-13) and the activity of systemic lupus erythematosus(systemic lupus, erythematosus, SLE) with antiphospholipid syndrome(antiphospholipid, syndrome, APS) the relationship between clinical characteristics and its value. Methods Using the residual collagen binding assay in 22 cases of SLE patients, 13 cases of SLE and APS(SLE-APS) patients and 22 patients with normal serum ADAMT-13 levels, and the relationship between SLE-APS patients and SLE patients with clinical manifestations, laboratory indexes, disease activity and prognosis. Statistical analysis using t test, 2 test and Spearman test.Results The serum ADAMTS-13 activity was decreased in patients with 1.SLE-APS, and correlated with the performance of thrombosis and thrombocytopenia. The activity of serum ADAMTS-13 in patients with 2.SLE-APS was inversely proportional to the activity of SLE. Conclusion SLE-APS patients may have more autoantibodies and the presence of thrombus and platelet showed more severe reduction may result in: von Willebrand factor(vWF) increase in the generation of the anti ADAMTS-13 antibody, which eventually lead to the decrease of serum ADAMTS-13 activity.