Increased von Willebrand factor levels in patients with systemic lupus erythematosus reflect inflammation rather than increased propensity for platelet activation.
Increased von Willebrand factor levels in patients with systemic lupus erythematosus reflect inflammation rather than increased propensity for platelet activation.
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DOI：
10.1136/lupus-2016-000162
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发表时间：
2016
影响因子：
3.9
通讯作者：
Eilertsen GØ
中科院分区：
文献类型：
--
作者：
Nossent JC;Raymond WD;Eilertsen GØ
血管性血友病因子 (VWF) 参与血小板栓子形成和蛋白质运输。系统性红斑狼疮 (SLE) 中 VWF 水平升高被认为是血管事件的危险因素。然而，VWF 蛋白水平并不能准确反映其血小板聚集功能，这一功能尚未在 SLE 中得到检测。对从区域狼疮登记处获得的 SLE 患者 (n=92) 的临床和实验室数据进行横断面研究。通过瑞斯托菌素诱导的血小板聚集（VWF 瑞斯托菌素辅因子，VWF:RCo）测定 VWF 功能，通过比浊法测定 VWF 水平（VWF 抗原，VWF:Ag）。每 VWF 单位的血小板聚集活性通过 VWF RCo/Ag 比率来估计。健康对照作为比较器，并通过非参数方法评估关联性。 SLE 患者的 VWF:Ag（142% vs 107%，p=0.001）和 VWF:RCo 水平（123% vs 78%，p<0.041）升高，但 VWF RCo/Ag 比率与对照组相似（0.83 vs 0.82，p=0.8）。在经历浆膜炎的患者中，VWF:Ag 水平较高，但与其他表现、血栓性疾病、系统性红斑狼疮疾病活动指数 2000 或系统性狼疮国际协作临床损伤指数无关。 VWF:Ag水平与VWF:RCo水平显着相关（Rs 0.8，p<0.001）、红细胞沉降率（ESR）（Rs 0.32，p<0.01）、抗dsDNA抗体（Rs 0.27，p<0.01）、总IgG（Rs 0.33，p<0.01）、纤维蛋白原（Rs 0.28，p<0.01）。 p<0.01）和铜蓝蛋白（Rs 0.367，p<0.01）水平。 VWF:RCo 水平与临床表现无关，但与 ESR、抗 dsDNA 和转铁蛋白水平相关。 VWF RCo/Ag 比率不存在血清学关联（全部 p>0.2）。在这个 SLE 队列中，VWF:Ag 的表现与急性期反应物相似，但 VWF:Ag 的增加与每单位 VWF 功能活动的增加并不匹配。因此，更多的 VWF 并不会增加 SLE 患者血小板聚集的倾向。
von Willebrand factor (VWF) is involved in platelet plug formation and protein transport. Increased VWF levels in systemic lupus erythematous (SLE) are considered risk factors for vascular events. VWF protein levels, however, do not accurately reflect its platelet-aggregating function, which has not been examined in SLE. Cross-sectional study with clinical and laboratory data obtained in patients with SLE (n=92) from a regional lupus registry. VWF function was determined by ristocetin-induced platelet aggregation (VWF ristocetin cofactor, VWF:RCo) and VWF levels by turbidimetric assay (VWF antigen, VWF:Ag). The platelet-aggregating activity per VWF unit was estimated by the VWF RCo/Ag ratio. Healthy controls served as comparators and associations were evaluated by non-parametric methods. VWF:Ag (142% vs 107%, p=0.001) and VWF:RCo levels (123% vs 78%, p<0.041) were increased in patients with SLE, but VWF RCo/Ag ratio was similar as in controls (0.83 vs 0.82, p=0.8). VWF:Ag levels were higher in patients experiencing serositis but unrelated to other manifestations, thrombotic disease, Systemic Lupus Erythematous Disease Activity Index 2000 or Systemic Lupus International Collaborative Clinics-Damage Index. VWF:Ag levels correlated significantly with VWF:RCo levels (Rs 0.8, p<0.001), erythrocyte sedimentation rate (ESR) (Rs 0.32, p<0.01), anti-dsDNA Ab (Rs 0.27, p<0.01), total IgG (Rs 0.33 p<0.01), fibrinogen (Rs 0.28, p<0.01) and ceruloplasmin (Rs 0.367, p<0.01) levels. VWF:RCo levels were not related to clinical findings but were correlated with ESR, anti-dsDNA and transferrin levels. No serological associations existed for VWF RCo/Ag ratio (all p>0.2). In this SLE cohort, VWF:Ag behaved similarly to acute-phase reactants, but VWF:Ag increases were not matched by increases in functional activity per unit of VWF. Thus, more VWF did not increase the propensity for platelet aggregation in SLE.