Abstract
BackgroundDespite expansion in the 2006 Sydney antiphospholipid syndrome (APS) classification criteria to include IgG/IgM anti-β2-glycoprotein (aβ2GPI) antibodies in addition to IgG/IgM anti-cardiolipin antibodies (aCL) and lupus anticoagulant (LAC), some individuals with clinical features of APS remain seronegative (seronegative APS or SNAPS) and are at risk of recurrent thrombosis and pregnancy morbidities. Our aim was to assess the value of “non-criteria” aPL antibodies to detect these SNAPS patients.MethodsOne hundred ninety-two APS patients, 90 SNAPS patients, 193 autoimmune disease controls, and 120 healthy controls were evaluated. Ten antiphospholipid antibodies (aPLs) were tested using commercial kits, including 5 non-criteria aPLs: anti-phosphatidylserine/prothrombin antibodies (aPS/PT) IgG/IgM, aCL IgA, aβ2GPI IgA, and anti-β2GPI Domain 1 (aβ2GPI-D1) IgG.ResultsUp to 60.9% of the SNAPS and 93.5% of APS patients were detected by at least one non-criteria aPL. aPS/PT IgG had the highest Youden index in classifying APS and SNAPS from controls. aPS/PT IgG and aβ2GPI Domain 1 IgG seem to be the most significant risk factors for thrombotic events and pregnancy morbidity, respectively. aPS/PT IgG/IgM and aβ2GPI-D1 IgG were detected in some SNAPS patients, while IgA isotypes of aCL/aβ2GPI tended to appear together with other biomarkers. The combined analysis showed enhanced diagnostic performance with the inclusion of non-criteria aPLs.ConclusionsRecognition of SNAPS patients is critical for clinical management and prevention of potential thrombotic and obstetric adverse events. The non-criteria antiphospholipid antibodies help to identify a considerable portion (60.9%) of these patients who otherwise may remain untreated and at clinical risk.
Highlights
Despite expansion in the 2006 Sydney antiphospholipid syndrome (APS) classification criteria to include IgG/IgM anti-β2-glycoprotein antibodies in addition to IgG/IgM anti-cardiolipin antibodies and lupus anticoagulant (LAC), some individuals with clinical features of APS remain seronegative and are at risk of recurrent thrombosis and pregnancy morbidities
seronegative APS (SNAPS) patients, just as classic APS patients, manifest increased risk for thrombotic events and pregnancy morbidities. These events may reoccur during the natural course of the disease [5] and in rare cases can result in a life-threatening thrombotic state leading to multi-organ dysfunction known as catastrophic antiphospholipid syndrome (CAPS) [6,7,8]
88 patients were classified as primary antiphospholipid syndrome (PAPS) and 104 patients as secondary antiphospholipid syndrome (SAPS) patients, of which 76 were comorbid with systemic lupus erythematosus (SLE), 25 with lupus-like disease, 2 with Sjogren’s syndrome, and 1 with rheumatoid arthritis
Summary
Despite expansion in the 2006 Sydney antiphospholipid syndrome (APS) classification criteria to include IgG/IgM anti-β2-glycoprotein (aβ2GPI) antibodies in addition to IgG/IgM anti-cardiolipin antibodies (aCL) and lupus anticoagulant (LAC), some individuals with clinical features of APS remain seronegative (seronegative APS or SNAPS) and are at risk of recurrent thrombosis and pregnancy morbidities. SNAPS patients, just as classic APS patients, manifest increased risk for thrombotic events and pregnancy morbidities These events may reoccur during the natural course of the disease [5] and in rare cases can result in a life-threatening thrombotic state leading to multi-organ dysfunction known as catastrophic antiphospholipid syndrome (CAPS) [6,7,8]. Increased recognition and a deeper understanding of APS have evolved with the development of assays for non-criteria aPLs and research into their role in the pathophysiology of APS. These non-criteria aPLs, which include anionic phospholipids, phospholipid-protein complexes, and plasma proteins, may help physicians to better manage suspected APS patients [9]. Research on anti-prothrombin (aPT) antibodies eventually led to the recognition of the importance of the complex of phosphatidylserine and prothrombin as a target for aPL antibodies
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