Tohidi-Esfahani, Ibrahim; Cohen, Hannah; Ioannou, Yiannis; Giles, Ian I; (2025) Pathogenesis of antiphospholipid antibody syndrome. In: Wallace, Daniel J and Hahn, Bevra Hannahs and Askanase, Anca and Crow, Mary K and Isenberg, David A and La Cava, Antonio and McMahon, Maureen A and Tsao, Betty P and Venuturupalli, Swamy and Weisman, Michael H, (eds.) Dubois' Lupus Erythematosus and Related Syndromes. (pp. 388-401). Elsevier: Amsterdam, The Netherlands.

Text Giles_Manuscript_Revised Dubois Pathogenesis of APS_2022_for upload.pdf Access restricted to UCL open access staff Download (371kB)

Abstract

Antiphospholipid syndrome (APS) is an acquired autoimmune thrombophilia characterized by venous, arterial, or small vessel thrombosis and/or obstetric morbidity associated with persistently positive antiphospholipid antibodies (aPLs). These aPLs are a heterogeneous family of autoantibodies that bind various phospholipids (principally cardiolipin) and phospholipid (PL)-binding serum proteins, the most important of these being β2-glycoprotein I (β2GPI). The aPLs that are currently used in clinical practice to identify patients with APS include detection of anticardiolipin antibodies and/or anti-β2GPI antibodies by enzyme-linked immunosorbent assay and/or positive lupus anticoagulant assay by prolongation of in vitro PL-dependent coagulation assays that can be corrected by addition of excess PL. The development of various aPL tests and evidence that aPLs have wide-ranging proinflammatory and procoagulant effects on target cells, complement factors, and regulators of the coagulation cascade to promote vascular thrombosis and/or pregnancy morbidity will be reviewed in this chapter.

Download activity - last month

Export as CSVJSONXML

Download activity - last 12 months

Export as XMLJSONCSV

Downloads by country - last 12 months

Export as CSVJSONXML

Archive Staff Only

View Item