title: Pathophysiological mechanisms in antiphospholipid syndrome.
abstract: Antiphospholipid syndrome is a systemic autoimmune disease associated with thrombosis and recurrent fetal loss in the setting of detectable antiphospholipid (aPL) antibodies. The major antigenic target has been identifed as β2-glycoprotein I (β2GPI), which mediates binding of aPL antibodies to target cells including endothelial cells, monocytes, platelets and trophoblasts, leading to prothrombotic and proinfammatory changes that ultimately result in thrombosis and fetal loss. This article summarizes recent insights into the role of β2GPI in normal hemostasis, interactions between aPL antibodies, β2GPI and cell-surface molecules, molecular prothrombotic and proinfammatory changes induced by aPL antibodies and pathogenic changes leading to fetal loss in antiphospholipid syndrome. New directions in therapy using these insights are examined.
authors.affiliation: Department of Internal Medicine, Division of Rheumatology, University of texas medical branch, Galveston, TX, USA.
authors.forename: Brock E
authors.initials: BE
authors.lastname: Harper
authors.forename: Rohan
authors.initials: R
authors.lastname: Wills
authors.forename: Silvia S
authors.initials: SS
authors.lastname: Pierangeli
keywords: annexin; anti-β2-glycoprotein I antibody; antiphospholipid antibodies; antiphospholipid syndrome; endothelial cell activation; pathogenesis; platelet activation; pregnancy loss; thrombosis; treatment