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血管性血友病因子及其裂解酶与系统性红斑狼疮的关系 被引量:2

The association of von Willebrand factor and von Willebrand factor-cleaving protease in systemic lupus erythematosus
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摘要 目的检测系统性红斑狼疮(SLE)患者血浆血管性血友病因子(VWF)水平及VWF裂解酶(VWF-CP)活性,探讨VWF及VWF-CP在SLE中的临床意义。方法采用残余胶原结合实验法及ELISA法分别对30例SLE患者血浆VWF-CP活性及VWF:AG水平进行检测。结果SLE患者血浆VWF:AG(114.6±16.3)%显著高于正常对照组(71.3±49.5)%(P<0.01),而血浆VWF-CP活性水平(57.7±16.3)%显著低于正常对照组(86.6±1.8)%(P<0.01),狼疮性肾炎(LN)组(130.1±40.6)%血浆VWF:AG水平显著高于非LN组(97.6±27.6)%(P<0.05),而VWF-CP活性显著降低(P<0.05),血浆VWF-CP活性水平与狼疮活动指数(SLEDAI)呈负相关(R=-0.4316,P<0.05)。18例肾活检的LN患者中,Ⅳ型VWF水平最高,VWF-CP活性最低,其余3型之间无差别。30例初发SLE患者综合治疗4周后血浆VWF:AG水平显著下降,VWF-CP活性水平在治疗后SLEDAI评分≥9分者,无显著性升高,SLEDAI评分<9分者,有显著性升高(P<0.05)。结论SLE的血浆VWF升高,VWF-CP活性低下,参与SLE尤其是LN的发生发展,内皮损伤和自身抗体可能是致SLE患者VWF升高,VWF-CP活性低下的原因。 Objective To evaluate the clinical significance of plasma yon Willebrand factor(vWF) and yon Willebrand factor-cleaving protease (vWF-CP) activity in systemic lupus erythematosus ( SLE ). Methods vWF antigen(vWF:Ag) and vWF-CP activity were respectively evaluated by using ELISA and residual-collagen binding assay( R-CBA ) in 30 patients with SLE and 40 normal controls. Results The level of the vWF: Ag in SLE patients (114. 6 ± 16. 3 )% was significanfly higher than that in the normal controls(71.3 ±49. 5)% (P 〈0.01) , while the level of plasma vWF-CP activity in the SLE patients(57.7 ± 16. 3 ) % was significantly lower than that in the conrols ( 86. 6 ± 1.8 ) % ( P 〈 0. 01 ). The level of vWF- CP activity was positively correlated with SLE disease activity index(SLEDAI). The alterations of these two indices were remarkable in lupus nephritis (LN) patients in comparison with those in non-LN patients. The level of vWF-CP activity in type IV LN was lowest among the four types of LN ( Ⅱ , Ⅲ, Ⅳ, Ⅴ ). The level of the vWF: Ag in 30 SLE patients was reduced four weeks after therapy, on the other hand, the vWF-CP activity was significantly increased in those with SLEDAI below 9, but unchanged in whose with SLEDAI above 9. Conclusions The injury of endothelial cells and to production autoantibodies against vWF-CP were the factors that resulted in lower vWF-CP activity in SLE patients and this lowered activity may influence the progression of SLE.
出处 《中华内科杂志》 CAS CSCD 北大核心 2006年第1期42-44,共3页 Chinese Journal of Internal Medicine
基金 国家自然科学基金资助项目(30470732)
关键词 红斑狼疮 系统性 von WILLEBRAND因子 von Willebrand病因子裂解酶 Lupus erythematosus, systemic yon Willebrand factor yon Willebrand diseases cleaving protease
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参考文献7

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同被引文献5

  • 1乐杰.妇产科学[M](第4版)[M].人民卫生出版社,1998.113-115.
  • 2Fujikawa K,Suzuki H,McMullen B,et al.Purification of human von Willebrand factor-cleaving protease and its identifi-cation as a new member of the metalloproteinase family[J].Blood, 2001,98:1662-1666.
  • 3Bombardier C,Gladman DD,Urowitz MB,et al.Derivation of the SLEDAI:a disease activity index for lupus patients.The Committee on Prognosis Studies in SLE[J].Arthritis Rheum,1992,35:630-640.
  • 4Rick ME,Moll S,Taylor MA.Clinieal use of rapid collagen binding assay for von Willebrand factor cleaving protease inpatients with thrombotic thrombocytopenic purpura[J].Thromb Haemost,2002,88:598-604.
  • 5孙云燕,杨玉英,朱媛媛,侯明.妊娠高血压综合征患者血浆VWF和PAI-1水平的变化及临床意义[J].现代妇产科进展,2003,12(3):194-196. 被引量:3

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