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Antiphospholipid syndrome, or antiphospholipid antibody syndrome (APS or APLS), is an autoimmune, hypercoagulable state caused by antiphospholipid antibodies.APS can lead to blood clots in both arteries and veins, pregnancy-related complications, and other symptoms like low platelets, kidney disease, heart disease, and rash.
Catastrophic antiphospholipid syndrome (CAPS), also known as Asherson's syndrome, is a rare autoimmune disease in which widespread, intravascular clotting causes multi-organ failure. [1] The syndrome is caused by antiphospholipid antibodies that target a group of proteins in the body that are associated with phospholipids .
In a suspected antiphospholipid syndrome, lupus anticoagulant is generally tested in conjunction with anti-apolipoprotein antibodies and anti-cardiolipin antibodies, and diagnostic criteria require one clinical event (i.e. thrombosis or pregnancy complication) and two positive blood test results spaced at least three months apart that detect at ...
21-hydroxylase antibodies Confirmed 0.93-1.4 per 10,000 [72] Autoimmune oophoritis: Ovaries: Anti-ovarian antibodies Probable Rare [73] Autoimmune orchitis: Testes: Anti-sperm antibodies Probable Rare [74] Autoimmune pancreatitis: Pancreas: IgG4, Anti-CA2 antibodies Confirmed 0.82-1.3 per 100,000 [75] Autoimmune polyendocrine syndrome type 1 (APS1)
Schematic representation of antibody. Anti-cardiolipin antibodies (ACA) are antibodies often directed against cardiolipin and found in several diseases, including syphilis, [1] antiphospholipid syndrome, livedoid vasculitis, vertebrobasilar insufficiency, Behçet's syndrome, [2] idiopathic spontaneous abortion, [3] and systemic lupus erythematosus (SLE). [4]
Livedoid vasculopathy has been linked to rheumatoid arthritis, systemic lupus erythematosus, scleroderma, polyarteritis nodosa, mixed and undifferentiated connective tissue diseases, and Sjogren's syndrome. Individuals with systemic lupus erythematosus who also have antiphospholipid antibody syndrome are more vulnerable. [9]
In the antiphospholipid antibody syndrome, the formation of the shield is disrupted by antibodies. Without the shield, there is an increased quantity of phospholipid molecules on cell membranes, speeding up coagulation reactions and causing the blood-clotting characteristic of the antiphospholipid antibody syndrome.
Thrombophilia (including inherited conditions such as factor V Leiden deficiency, protein C or S deficiency, or antiphospholipid antibody syndrome) is another common cause. [3] Nearly one-third of patients have a myeloproliferative disorder (e.g. polycythemia vera [ 6 ] or primary thrombocytosis), most commonly due to a Janus kinase 2 (JAK2 ...
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