Antiphospholipid antibodies (aPL) are a heterogeneous family of antibodies, including those specific for a variety of phospholipid (PL)-binding proteins and also those reacting with PL molecules. The former seem to be associated with the antiphospholipid syndrome (APS). At present, the main proteins proposed as antigens are β2 glycoprotein I, prothrombin, protein C, protein S, kininogens and annexin V. Anionic PL might play a key role “in vivo” in the binding of aPL to PL-bound proteins. Different mechanisms may be involved in the pathogenesis of the APS, including effects of aPL on the protein C system and antithrombin III and also on platelets, endothelial cells and monocytes. Recent data on experimental animal models have provided support for a causative role of aPL in the clinical complications of the APS.

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