Nusinow S R, Federici A B, Zimmerman T S, Curd J G
Arthritis Rheum. 1984 Dec;27(12):1405-10. doi: 10.1002/art.1780271211.
The plasma concentrations of von Willebrand factor antigen (vWF:Ag) were determined in 101 patients who had the following diagnoses: vasculitis 8 patients, systemic lupus erythematosus (SLE) 51, rheumatoid arthritis (RA) 28, asthma 7, hereditary angioedema 7. The greatest mean concentration of vWF:Ag, 469% (normal 100% +/- 50), was observed in patients with vasculitis, often without elevation of the erythrocyte sedimentation rate. The mean concentration of vWF:Ag was also increased in both SLE (277%) and RA (194%). Twenty-four patients (15 with SLE, 6 with vasculitis, 3 with RA) had vWF:Ag concentrations greater than 300%. Four of these patients died within 1 year of the date of the study. Of the 15 SLE patients, 9 had vasculitis and 2 had active glomerulonephritis. The 3 RA patients had severe disease associated with extraarticular manifestations. Elevated vWF:Ag may reflect vascular damage, while markedly elevated levels of vWF:Ag appear to indicate a poor prognosis.
对101例确诊患者测定了血管性血友病因子抗原（vWF:Ag）的血浆浓度，这些患者的诊断如下：血管炎8例、系统性红斑狼疮（SLE）51例、类风湿关节炎（RA）28例、哮喘7例、遗传性血管性水肿7例。血管炎患者中观察到vWF:Ag的平均浓度最高，为469%（正常为100%±50），且红细胞沉降率通常无升高。SLE（277%）和RA（194%）患者的vWF:Ag平均浓度也有所升高。24例患者（15例SLE、6例血管炎、3例RA）的vWF:Ag浓度大于300%。其中4例患者在研究日期后1年内死亡。15例SLE患者中，9例有血管炎，2例有活动性肾小球肾炎。3例RA患者有与关节外表现相关的严重疾病。vWF:Ag升高可能反映血管损伤，而vWF:Ag水平显著升高似乎表明预后不良。
