[Antiphospholipid syndrome]

Since 1987 the antiphospholipid syndrome has been recognized as a major cause of acquired thrombophilia, whether it is associated with systemic lupus erythematosus or occurs as a free-standing syndrome (primary form). This autoimmune condition associates in young patients recurrent thrombosis (both venous and/or arterial) and/or a variety of obstetric complications with the persistent presence of antiphospholipid antibodies (aPL). These traditionally comprise anticardiolipin antibodies and lupus anticoagulants, respectively detected by immunological and clotting tests. Despite their name aPL do not bind to phospholipids per se, but are directed at phospholipid-binding plasma proteins, especially beta 2-glycoprotein I and prothrombin. Because the risk of recurrence is high, the standard of care is prolonged and high-intensity warfarin (INR near 3) after a venous thromboembolic event, together with the management of associated vascular risk factors. Prevention of adverse obstetric outcomes is frequently achieved by a combination of low-dose aspirin and heparin.