Syndrome Antiphospholipid or sometimes called Antiphospholipid syndrome (APS) as an arterial or venous thrombosis with recurrent, abortion, accompanied by anti-cardiolipin or lupus anticoagulant test positive for disease continued. The disorder can be secondary to systemic lupus erythematosus or other autoimmune diseases, but may occur alone (primary antiphospholipid syndrome). Regardless of primary or secondary to APS, the clinical manifestations and laboratory features, and non-discriminatory.
Although the disease more common in adults, but children also occur. Female incidence rate than men. APS's family orientation is not obvious, but the relatives of patients with anti-cardiolipin or lupus anticoagulant checking often can be positive.
The etiology and pathogenesis. As the crowd of anti-phospholipid antibody-positive patients, only part of the clinical manifestations, and therefore the occurrence of APS with other factors. How do antiphospholipid antibodies cause thrombosis is still unclear. Some studies suggesting that these antibodies can inhibit catalysis by phospholipid with a negative charge of the blood clotting cascade. These reactions include the activation of factor X, prothrombin - thrombin conversion, protein C activation and activated protein C due to the inactivation of factors Va. Antiphospholipid antibodies, or in protein C activation and the role of factor Va inactivation, cause a patient in a "prothrombotic state." Has proven anticardiolipin antibodies and phosphatidylserine cross-reactivity with those of platelet binding and activate platelets, may also cause thrombosis. Other possible mechanisms include increased platelet synthesis of thromboxane, inhibit prostaglandin synthesis, and stimulation of tissue factor by endothelial cells.
The etiology and pathogenesis. As the crowd of anti-phospholipid antibody-positive patients, only part of the clinical manifestations, and therefore the occurrence of APS with other factors. How do antiphospholipid antibodies cause thrombosis is still unclear. Some studies suggesting that these antibodies can inhibit catalysis by phospholipid with a negative charge of the blood clotting cascade. These reactions include the activation of factor X, prothrombin - thrombin conversion, protein C activation and activated protein C due to the inactivation of factors Va. Antiphospholipid antibodies, or in protein C activation and the role of factor Va inactivation, cause a patient in a "prothrombotic state." Has proven anticardiolipin antibodies and phosphatidylserine cross-reactivity with those of platelet binding and activate platelets, may also cause thrombosis. Other possible mechanisms include increased platelet synthesis of thromboxane, inhibit prostaglandin synthesis, and stimulation of tissue factor by endothelial cells.
In the anti-phospholipid antibody-mediated thrombosis process, called β2-glycoprotein 1 (β2GP1) of plasma proteins popular attention. β2GP1 can be combined with a negative charge such as the phospholipid molecules, as β2GP1 suppress the negative charge with phospholipids catalyzed by the clotting reaction, such as the prothrombin - thrombin conversion, so it is considered to be "natural anticoagulant." Mice immunized with the same time in order to β2GP1 production of anti-β2GP1 and anti-cardiolipin antibodies. In addition to β2GP1, phospholipid-binding proteins including human IgG antiphospholipid antibodies can also induce anti-cardiolipin antibodies. Antiphospholipid antibody currently considered by the role of anticoagulation during and β2GP1 cause thrombosis.
