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Abstract
Antiphospholipid antibodies (aPL) constitute a heterogeneous group of autoantibodies that share the ability to bind phospholipids (PL) alone, protein-PL complexes, or PL-binding proteins. They have been detected in isolation, in association with autoimmune diseases such as systemic lupus erythematosus (SLE), and during the course of different infections. aPL have been associated with an array of clinical manifestations in virtually every organ, although deep vein and arterial thrombosis as well as pregnancy morbidity are predominant. The co-occurrence of these clinical findings with aPL constitutes the so-called antiphospholipid syndrome (APS).
aPL can be detected by immunological methods [e.g., anticardiolipin antibodies (aCL)] or by functional methods that exploit the effect of aPL on blood coagulation [lupus anticoagulant (LA)]. Since aPL are heterogeneous, numerous immunological and coagulation assays have been developed. These assays have not been fully standardized, and, therefore, problems such as high interlaboratory variation are relatively frequent. Recently, recommendations have been published regarding LA and aCL testing.
Not all aPL are pathogenic. However, when they are not associated with infections, they have a role in the pathogenesis of APS. Clinical and experimental data have shown that aPL exert their pathogenic activity by interfering with the function of coagulation factors, such as thrombin and factors X, XI and XII, and with the function of anticoagulant proteins of the protein C system. In addition, aPL interaction with platelets and endothelial cells induces a pro-adhesive activated phenotype.
| Abbreviations: | ||
| aCL | = | anticardiolipin antibodies |
| anti-PS/PT | = | anti-phosphatidylserine/ prothrombin antibodies |
| anti-PT | = | anti-prothrombin antibodies |
| anti-β 2GPI | = | anti-β 2 glycoprotein I antibodies |
| APCR | = | activated protein C resistance |
| aPL | = | antiphospholipid antibodies |
| APC | = | activated protein C |
| APS | = | antiphospholipid syndrome |
| aPTT | = | activated partial thromboplastin time |
| β2GPI | = | β2 glycoprotein I |
| cPLA2 | = | cytosolic phospholipase A2 |
| CL | = | cardiolipin |
| CV | = | coefficient of variation |
| dPT | = | dilute prothrombin time |
| dRVVT | = | dilute Russel Viper Venom Test |
| EC | = | endothelial cells |
| ELISA | = | enzyme-linked immunosorbent assay |
| EPCR | = | endothelial protein C receptor |
| HMWK | = | high molecular weight kininogen |
| HUVEC | = | human umbilical vein endothelial cells |
| ICAM | = | intracellular adhesion molecule |
| IL | = | interleukin |
| KCT | = | Kaolin Clotting Time |
| LA | = | lupus anticoagulant |
| MAPK | = | mitogen-associated protein kinase |
| MCPI | = | monocyte chemoattractant protein I |
| NFκ B | = | nuclear factor κB |
| PAI-1, PAI-2 | = | plasminogen activator inhibitors 1, 2 |
| PK | = | prekallikrein |
| PL | = | phospholipid |
| PS | = | phosphatidylserine |
| PT | = | prothrombin |
| RIA | = | radioimmunoassay |
| SLE | = | systemic lupus erythematosus |
| SSC/ISTH | = | Scientific and Standardization Committee of the International Society of Thrombosis and Haemostasis |
| TF | = | tissue factor |
| TFPI | = | tissue factor pathway inhibitor |
| TNF | = | tumor necrosis factor |
| t-PA | = | tissue-type plasminogen activator |
| TXB2 | = | thromboxane B2 |
| u-PA | = | urokinase-type plasminogen activator |
| VCAM | = | vascular cell adhesion molecule |
| Abbreviations: | ||
| aCL | = | anticardiolipin antibodies |
| anti-PS/PT | = | anti-phosphatidylserine/ prothrombin antibodies |
| anti-PT | = | anti-prothrombin antibodies |
| anti-β 2GPI | = | anti-β 2 glycoprotein I antibodies |
| APCR | = | activated protein C resistance |
| aPL | = | antiphospholipid antibodies |
| APC | = | activated protein C |
| APS | = | antiphospholipid syndrome |
| aPTT | = | activated partial thromboplastin time |
| β2GPI | = | β2 glycoprotein I |
| cPLA2 | = | cytosolic phospholipase A2 |
| CL | = | cardiolipin |
| CV | = | coefficient of variation |
| dPT | = | dilute prothrombin time |
| dRVVT | = | dilute Russel Viper Venom Test |
| EC | = | endothelial cells |
| ELISA | = | enzyme-linked immunosorbent assay |
| EPCR | = | endothelial protein C receptor |
| HMWK | = | high molecular weight kininogen |
| HUVEC | = | human umbilical vein endothelial cells |
| ICAM | = | intracellular adhesion molecule |
| IL | = | interleukin |
| KCT | = | Kaolin Clotting Time |
| LA | = | lupus anticoagulant |
| MAPK | = | mitogen-associated protein kinase |
| MCPI | = | monocyte chemoattractant protein I |
| NFκ B | = | nuclear factor κB |
| PAI-1, PAI-2 | = | plasminogen activator inhibitors 1, 2 |
| PK | = | prekallikrein |
| PL | = | phospholipid |
| PS | = | phosphatidylserine |
| PT | = | prothrombin |
| RIA | = | radioimmunoassay |
| SLE | = | systemic lupus erythematosus |
| SSC/ISTH | = | Scientific and Standardization Committee of the International Society of Thrombosis and Haemostasis |
| TF | = | tissue factor |
| TFPI | = | tissue factor pathway inhibitor |
| TNF | = | tumor necrosis factor |
| t-PA | = | tissue-type plasminogen activator |
| TXB2 | = | thromboxane B2 |
| u-PA | = | urokinase-type plasminogen activator |
| VCAM | = | vascular cell adhesion molecule |