Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by frequent clotting in arteries and veins and/or miscarriages. Autoantibodies to phospholipids and to beta 2 glycoprotein 1 (β(2)GP1) play an important role in the pathogenesis of APS. Antibodies to the domain 1 of β(2)GP1 (β(2)GP1-D1) have been suggested as a risk marker for thrombosis and to a lesser extent for pregnancy complications in patients suffering from APS. Despite significant interest in anti-β(2)GP1-D1 antibodies and a considerable research history, the number of studies is still limited and acceptance of the clinical significance of this biomarker is still evolving. The present review summarizes the current knowledge of anti-β(2)GP1-D1 antibodies and provides insights on recent discoveries. Moreover, we present a suggested guideline for future studies to better understand and verify the clinical utility of anti-β(2)GP1-D1 antibodies.
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