title: [The association of von Willebrand factor and von Willebrand factor-cleaving protease in systemic lupus erythematosus].
abstract: OBJECTIVE
  To evaluate the clinical significance of plasma von Willebrand factor (vWF) and von 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 significantly 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 controls (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.
authors.affiliation: Department of Nephrology, First Affiliated Hospital of Soochow University, Suzhou, Jiangsu 215006, China.
authors.forename: Guo-yuan
authors.initials: GY
authors.lastname: Lu
authors.forename: Xiao-fang
authors.initials: XF
authors.lastname: Guo
authors.forename: Zhao-yue
authors.initials: ZY
authors.lastname: Wang
authors.forename: Xia
authors.initials: X
authors.lastname: Bai
authors.forename: Jian
authors.initials: J
authors.lastname: Su
authors.forename: Chang-gen
authors.initials: CG
authors.lastname: Ruan
mesh_terms: D051722:ADAM Proteins / Q000097:blood*; D000071120:ADAMTS13 Protein; D000328:Adult; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000097:blood* / Q000201:enzymology*; D008297:Male; D008875:Middle Aged; D014841:von Willebrand Factor / Q000378:metabolism*
chemical_list: D014841:von Willebrand Factor; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human