Trends in the Antiphospholipid Syndrome Criteria

Abstract

Copyright: © 2013 Forastiero R. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. The Antiphospholipid Syndrome (APS) is an autoimmune disease characterized clinically by the occurrence of either venous or arterial thrombosis in different vascular beds, and/or recurrent miscarriages in the first trimester, or fetal death in the second or third trimesters, or severe pre-eclampsia requiring delivery of a premature infant before 34 weeks of gestation. Cerebrovascular infarction is the most common clinical feature within the arterial circulation, whereas lower limb deep venous thrombosis and pulmonary embolism are the main locations within the venous circulation [1]. The detection of persistently elevated levels of antiphospholipid antibodies (aPL) is a requisite laboratory feature for the diagnosis of APS. The positivity for at least one aPL test: lupus anticoagulant (LA) by clotting assays and/or IgG/IgM anticardiolipin (aCL) and/ or IgG/IgM anti-β2 glycoprotein I antibodies (aβ2GPI) by Enzyme-Linked Immunosorbent (ELISA) assays must be detected. aPL must be found on two or more occasions at least 12 weeks apart. In the 2006 updated APS criteria it is advised to classify APS patients in studies into one of the following categories: I, more than one laboratory criteria present (any combination); IIa, LA present alone; IIb, aCL present alone; IIc, aβ2GPI present alone [2]. aPL can occur in isolation or in association with other autoimmune conditions, particularly Systemic Lupus Erythematosus (SLE). The catastrophic APS is a rare variant of APS characterized by microthrombi in multiple organs. It is commonly triggered by several factors including infection, trauma, surgery, and the withdrawal of oral anticoagulation. Less than 1% of patients with the APS develop the catastrophic complication but it has a life-threatening clinical course. Sometimes aPL coagulopathy may start with a hemorrhagic syndrome when a severe thrombocytopenia, or an acquired thrombocytopathy, factor VIII inhibitor, or prothrombin deficiency is present.