Abstract
Objectives: This study aimed to investigate the association between plasma von Willebrand factor (VWF) level, ADAMTS13 activity, and neuroimaging features of cerebral small vessel disease (CSVD), including the CSVD neuroimaging markers and the overall CSVD burden.Methods: CSVD patients admitted to our hospital from 2016 to 2020 were recruited. Plasma VWF level and ADAMTS13 activity were measured. The overall effect of CSVD on the brain was described as a validated CSVD score. We evaluated the association between VWF levels, ADAMTS13 activity, and the increasing severity of CSVD score by the logistic regression model.Results: We enrolled 296 patients into this study. The mean age of the sample was 69.0 years (SD 7.0). The mean VWF level was 1.31 IU/mL, and the ADAMTS13 activity was 88.01 (SD 10.57). In multivariate regression analysis, lower ADAMTS13 activity and higher VWF level was related to white matter hyperintensity (WMH) [β = −7.31; 95% confidence interval (CI) (−9.40, −4.93); p<0.01; β = 0.17; 95% confidence interval (0.11, 0.23); p<0.01], subcortical infarction (SI) [(β = −9.22; 95% CI (−11.37, −7.06); p<0.01); β = 0.21; 95% confidence interval (0.15, 0.27); p<0.01] independently, but not cerebral microbleed (CMB) [(β = −2.3; 95% CI (−4.95, 0.05); p = 0.22); β = 0.02; 95% confidence interval (−0.05, 0.08); p = 0.63]. Furthermore, ADAMTS13 activity was independently negatively correlated with the overall CSVD burden (odd ratio = 21.33; 95% CI (17.46, 54.60); p < 0.01) after adjustment for age, history of hypertension, and current smoking.Conclusions: Reducing ADAMTS13 activity change is related to white matter hyperintensity, subcortical infarction, but not with cerebral microhemorrhage. In addition, ADAMTS13 may have played an essential role in the progression of CSVD.
Highlights
Cerebral small vessel disease (CSVD) is a syndrome with various structural or functional lesions involving perforating vessels that leads to parenchymal injury, which causes clinical, cognitive, neuroimaging, and neuropathological manifestation
A total of 296 patients with CSVD were included in this study, of which 125 were male
Subcortical lesions (SI) accounted for 42.3% (n = 125) of the total sample, cerebral microbleeds (CMB) for 28.0% (n = 83), and high white matter hyperintensity (WMH) (Fazekas score of 2–3) for 41.6% (n = 123)
Summary
Cerebral small vessel disease (CSVD) is a syndrome with various structural or functional lesions involving perforating vessels that leads to parenchymal injury, which causes clinical, cognitive, neuroimaging, and neuropathological manifestation. The pathogenesis of the CSVD is not well known, but more evidence suggests that endothelial dysfunction is a crucial link leading to changes in cerebrovascular structure and function in patients with CSVD (Wardlaw et al, 2013; Jackman and Iadecola, 2015). For CSVD, the related markers such as endothelial cell injury, inflammatory response, and coagulation/coagulation markers are well studied, and the connection between homocysteine, asymmetric dimethylarginine (ADMA), von Willebrand factor (VWF), and CSVD has been widely recognized (Wang et al, 2016; Janes et al, 2019; Nam et al, 2019). A disintegrin and metalloproteinase with a thrombospondin motif repeat 13 (ADAMTS13) regulate the activity of VWF by cutting ultra-long VWF multimers into smaller, less active molecules and exert its anti-inflammatory and antithrombotic properties (Gerritsen et al, 2001)
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