Abstract
The specific value of IgA Anti-β2glycoprotein I antibodies (aB2GP1) in the diagnosis and management of antiphospholipid syndrome (APS) is still controversial and a matter of active debate. The relevance of the IgA aB2GP1 isotype in the pathophysiology of APS has been increasingly studied in the last years. There is well know that subjects with multiple positive APS tests are at increased risk of thrombosis and/or miscarriage. However, these antibodies are not included in the 2006 APS classification criteria. Since 2010 the task force of the Galveston International Congress on APS recommends testing IgA aB2GP1 isotype in patients with APS clinical criteria in the absence of criteria antibodies. In this review, we summarize the molecular and clinical “state of the art” of the IgA aB2GP in the context of APS. We also discuss some of the characteristics that may help to evaluate the real value of the IgA aB2GP1 determination in basic research and clinical practice. The scientific community should be aware of the importance of clarifying the role of IgA aB2GP1 in the APS diagnosis.
Highlights
Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by the occurrence of thrombotic events or gestational morbidity in subjects with antiphospholipid antibodies [1]
The APS was classified into three different categories: (1) Primary APS (P-APS) when APS was not associated with any other autoimmune disease, (2) systemic autoimmune diseases (SAD) associated APS (SAD-APS) when the diagnosis of APS was performed in the context of autoimmune disease and (3) Catastrophic APS (C-APS) [4,5]
ACL: Anti-cardiolipin antibodies, anti B2GP1 (aB2GP1): Anti-Beta 2 Glycoprotein I Antibodies, ELISA: Enzyme-linked immunosorbent assays, in vitro fertilization treatment (IVF): In vitro fertilization treatment, P-APS: Primary Antiphospholipid syndrome not associated with other entities, SAD-APS: Antiphospholipid syndrome associated with Systemic autoimmune diseases SN-APS: Seronegative Antiphospholipid syndrome
Summary
Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by the occurrence of thrombotic events or gestational morbidity in subjects with antiphospholipid antibodies (aPL) [1]. Laboratory criteria include the positivity of any aPL: Lupus anticoagulant (LA), anti-cardiolipin (aCL), or anti B2GP1 (aB2GP1) antibodies of IgM or IgG isotypes. Hughes and Khamashta described a group of patients with APS clinical features that were persistently negative for criteria aPL. Many SN-APS patients are positive for antibodies not included in the classification criteria (non-criteria aPL) [9,10]. Since 2010, the task force of the Galveston International Congress on APS recommends testing IgA isotype in patients with APS clinical criteria with persistently negative results for criteria aPL [15]. We discuss some methodological and technical limitations, challenges, and drawbacks that may be considered for the interpretation of these results (Table 2) These considerations would help both physicians and researchers to better elucidate the value of IgA aB2GP1 tests in different scenarios. P-APS: Primary Antiphospholipid syndrome without associating with other entities
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