Antiphospholipid syndrime: Pathogenesis, Diagnosis & Treatments
Pathogenesis
Antiphospholipid syndrome is an autoimmune, hypercoagulable state caused by an antiphospholipid antibody. APS causes thrombosis in the arteries and veins, and may cause miscarriage, premature birth, severe preeclampsia, etc. in connection with pregnancy. Antiphospholipid Ab includes anti-cardiolipin Ab and Lupus Anticoagulant, which bind to the phospholipid in the plasma membrane. Anti-ApoH and anti-cardiplipin Ab inhibit protein C and glycoprotein, which bind to ApoH and degrade factor V, resulting in hypercoagulable state. Lupus anticoagulant Ab binds to prothrombin and promotes the separation into thrombin. Lupus anticoagulant Ab also targets β2glycoprotein 1, which is more associated with thrombosis and has a higher thrombus risk when LA (Lupus anticoagulant) and moderate to high titer (> 40 GPLU) anticardiolipin Ab together. In addition, the antiphospholipid antibody causes decreased trophoblast viability, syntialization, and invasion, which may interfere with the production of hormones and signaling molecules generated from trophoblasts, resulting in complications related to pregnancy. The risk factors for primary APS (without underlying disesase) include HLA-DR7, secondary autoimmune diseases such as HLA-B8, HLA-DR2 and HLA-DR3.
Diagnosis
Diagnosis is made when one or more clinical criteria and one or more laboratory criteria are met. The clinical criteria are venous, arterial, or small vessel thrombosis with no significant evidence of inflammation in the vessel wall, It is considered to be satisfied when one occurrence of miscarriage or one occurrence of preterm birth before 34 weeks of gestation or three or more gestational sacrifice occurs before 10 weeks. In laboratory criteria, anti-cardioplipin IgG / IgM or anti-β2 glycoprotein IgG / IgM were detected in non-cofactor dependent ELISA more than once every 12 weeks with medium to high titer (> 99%) or lupus anticoagulant And more than two times. Types of APS include primary APS, secondary APS, and catstrophic APS according to the presence or absence of underlying disease. CAPS is diagnosed when vascular thrombosis is present in three or more organs or tissues, symptoms occur within a week, small vessel thrombosis is present, and antiphospholipid Ab is present.
Treatments
Aspirin is administered to prevent platelet activation if there is a high antibody titer but there is no thrombotic event (clinical event). In case of thrombotic event, warfarin is used to maintain INR 2.0 ~ 3.0. In case of thrombocytopenia, high dose steroid IV immunoglobulin, danazol, cyclosporine, and dapsone. In pregnant women, low molecular weight heparin and low-dose aspirin are used because of the teratogenicity of warfarin. If you are not responding to treatment, you can try plasmapheresis.
Antiphospholipid syndrome is an autoimmune, hypercoagulable state caused by an antiphospholipid antibody. APS causes thrombosis in the arteries and veins, and may cause miscarriage, premature birth, severe preeclampsia, etc. in connection with pregnancy. Antiphospholipid Ab includes anti-cardiolipin Ab and Lupus Anticoagulant, which bind to the phospholipid in the plasma membrane. Anti-ApoH and anti-cardiplipin Ab inhibit protein C and glycoprotein, which bind to ApoH and degrade factor V, resulting in hypercoagulable state. Lupus anticoagulant Ab binds to prothrombin and promotes the separation into thrombin. Lupus anticoagulant Ab also targets β2glycoprotein 1, which is more associated with thrombosis and has a higher thrombus risk when LA (Lupus anticoagulant) and moderate to high titer (> 40 GPLU) anticardiolipin Ab together. In addition, the antiphospholipid antibody causes decreased trophoblast viability, syntialization, and invasion, which may interfere with the production of hormones and signaling molecules generated from trophoblasts, resulting in complications related to pregnancy. The risk factors for primary APS (without underlying disesase) include HLA-DR7, secondary autoimmune diseases such as HLA-B8, HLA-DR2 and HLA-DR3.
Diagnosis
Diagnosis is made when one or more clinical criteria and one or more laboratory criteria are met. The clinical criteria are venous, arterial, or small vessel thrombosis with no significant evidence of inflammation in the vessel wall, It is considered to be satisfied when one occurrence of miscarriage or one occurrence of preterm birth before 34 weeks of gestation or three or more gestational sacrifice occurs before 10 weeks. In laboratory criteria, anti-cardioplipin IgG / IgM or anti-β2 glycoprotein IgG / IgM were detected in non-cofactor dependent ELISA more than once every 12 weeks with medium to high titer (> 99%) or lupus anticoagulant And more than two times. Types of APS include primary APS, secondary APS, and catstrophic APS according to the presence or absence of underlying disease. CAPS is diagnosed when vascular thrombosis is present in three or more organs or tissues, symptoms occur within a week, small vessel thrombosis is present, and antiphospholipid Ab is present.
Treatments
Aspirin is administered to prevent platelet activation if there is a high antibody titer but there is no thrombotic event (clinical event). In case of thrombotic event, warfarin is used to maintain INR 2.0 ~ 3.0. In case of thrombocytopenia, high dose steroid IV immunoglobulin, danazol, cyclosporine, and dapsone. In pregnant women, low molecular weight heparin and low-dose aspirin are used because of the teratogenicity of warfarin. If you are not responding to treatment, you can try plasmapheresis.
