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Essentials    The antiphospholipid syndrome predisposes to thrombosis due to activation of endothelium and blood components.  The role of anti‐β2GPI antibodies in VWF release and ADAMTS13 function is not well understood.  Some anti‐β2GPI antibodies induce endothelial release of soluble VWF but not VWF strings.  An anti‐β2GPI antibody can decrease ADAMTS13 activity in vitro similar to ex vivo results.Background  Antiphospholipid syndrome ( APS ) is characterized by recurrent thromboembolic events in the setting of pathologic autoantibodies, some of which are directed to β2‐Glycoprotein 1 (β2 GPI ). The mechanisms of thrombosis in  APS  appear to be multifactorial and likely include a component of endothelial activation. Among other things, activated endothelium secretes von Willebrand factor, a hemostatic protein that in excess can increase the risk of thrombosis.    Objective  We hypothesized that anti‐β2 GPI  antibodies could regulate the release and modulation of  VWF  from endothelial cells.    Patients/Methods  Isolated anti‐β2 GPI  antibodies from patients with  APS  were assayed for their ability to induced  VWF  release from  HUVEC s and modulate the effects of  ADAMTS 13 in a shear‐dependent assay.    Results  We observed that anti‐β2 GPI  antibodies from some patients with  APS  induced  VWF  release from human endothelial cells but did not induce formation of cell‐anchored  VWF ‐platelet strings. Finally, we also determined that one of the Anti‐β2 GPI  antibodies tested can inhibit the function of  ADAMTS 13, the main modulator of extracellular  VWF .    Conclusions  These results suggest that  VWF  and  ADAMTS 13 may play a role in the prothrombotic phenotype of  APS .

Effects of anti‐β2 GPI antibodies on VWF release from human umbilical vein endothelial cells and ADAMTS 13 activity