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Antiphospholipid syndrome is known for causing arterial or venous blood clots, in any organ system, and pregnancy-related complications.While blood clots and pregnancy complications are the most common and diagnostic symptoms associated with APS, other organs and body parts may be affected like platelet levels, heart, kidneys, brain, and skin.
Graham Hughes is a rheumatologist. He discovered antiphospholipid syndrome , which was named Hughes syndrome after him, and opened Europe's first lupus clinic. [ 1 ] [ 2 ] He is editor of the journal Lupus , and has published ten books on lupus and related conditions.
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 .
Antiphospholipid syndrome was described in full in the 1980s, after various previous reports of specific antibodies in people with systemic lupus erythematosus and thrombosis. [18] [49] The syndrome is often attributed to the British rheumatologist Graham R.V. Hughes, and is often referred to as Hughes syndrome for that reason. [50]
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 ...
Treatment usually consists of a daily low dose (80–100 mg) of aspirin. [13] Anticoagulants (e.g. warfarin) or antiplatelets (clopidogrel) are often additionally prescribed following formation of a medically significant clot. Thrombelastography is more commonly being used to diagnose hypercoagulability and monitor anti-platelet therapy.
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 ...
Hughes–Stovin syndrome (HSS) is a rare autoimmune disorder often described as inflammation in relation to blood vessels, a form of vasculitis. It is not associated with any known cause and is typically characterized by multiple aneurysms in pulmonary arteries and deep vein thromboses .