Recent developments in our understanding of the antiphospholipid syndrome

The antiphospholipid syndrome is an autoimmune disease that manifests clinically as recurrent thrombotic complications or foetal losses and serologically with elevated levels of antiphospholipid antibodies in the plasmas of these patients. The term 'antiphospholipid syndrome' is confusing, because the auto-antibodies are not directed against phospholipids but towards a plasma protein, β(2) -glycoprotein I. For many years, the reason why auto-antibodies against β(2) -glycoprotein I were pro-thrombotic was unclear, because β(2) -glycoprotein I seems to be an obsolete protein in our circulation. Human and mice deficient in this protein do not express a clear phenotype. Recent studies on the structure and function of β(2) -glycoprotein I have provided novel insights into the importance of this protein in physiology and its role in the pathology of the antiphospholipid syndrome.