Pathophysiology of thrombotic APS: where do we stand?

The antiphospholipid syndrome (APS) is diagnosed when patients with thrombotic complications or foetal losses have elevated levels of antiphospholipid antibodies in their plasmas. The term APS is confusing, because the pathogenic auto-antibodies are not directed against phospholipids but towards a plasma protein, beta(2)-glycoprotein I. For many years the reason why auto-antibodies against beta(2)-glycoprotein I were pro-thrombotic was unclear, because man and mice deficient in beta(2)-glycoprotein I do not express a clear phenotype. Animal models in which passive transfer of patient antibodies into mice resulted in an increased thrombotic response have provided novel insights in the importance of this protein in the pathology of APS. Lupus (2012) 21, 704-707.