antiphospholipid antibody syndrome

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Related to Antiphospholipid syndrome: Hughes syndrome, Antiphospholipid antibody syndrome

antiphospholipid antibody syndrome

A thrombophilic disorder in which venous or arterial thrombosis, or both, may occur. The serologic markers are antiphospholipid antibodies (anticardiolipin antibodies), the lupus anticoagulant, or both; APAS is characterised by the presence of circulating antiphospholipid antibodies (APA), in particular against cardiolipin (ACA), which overlap with lupus anticoagulants.

APAs occur in patients with SLE and are associated with thromboembolic phenomena, often accompanied by habitual abortion, with mid-pregnancy foetal wastage due to thrombosis of placental vessels, DVT, recurrent MI (coronary thrombosis), pulmonary HTN, thrombocytopaenia, and occasionally also renal infarction. Other manifestations include livido reticularis, valvular heart disease, labile HTN, a positive Coombs test and recurrent digital gangrene, often in a background of SL; thrombosis may be related to the antibodies that inhibit prostacyclin (PGI2) production and interfere with the release of arachidonic acid from the cell membrane. The association of recurrent thromboses (cerebral), repeated spontaneous abortions and renal disease, often in ANA-negative SLE patients, has been termed the circulating lupus anticoagulant syndrome, which may be accompanied by repeated foetal wastage and IgM gammopathy.

Neurologic dysfunction may be accompanied by amaurosis fugax, CVAs, chorea, epilepsy, Guillain-Barré syndrome, migraines, multiple sclerosis-like disease, myelopathy and TIAs. APAs/ACAs may cause disease by acting on platelet membranes or vascular endothelia; APAs occur in TIAs and cardiac valve replacement.

High titres (> 7 standard deviations of IgG ACA) are reported as having 80% specificity for this condition. APAs cross-react with DNA, explaining the biological false positive serological test for syphilis commonly seen in SLE; increased APAs are found at autopsy in patients with thromboembolism.
 
Management
Long-term anticoagulation with warfarin, with INR ≥ 3.

antiphospholipid antibody syndrome

Antiphospholipid syndrome, circulating lupus anticoagulant syndrome Immunology The association of recurrent thromboses–cerebral, repeated spontaneous abortions and renal disease often in ANA-negative SLE Pts, which may be accompanied by repeated fetal wastage and IgM gammopathy; APA has been defined as a ' thrombophilic disorder in which venous or arterial thrombosis, or both, may occur. The serologic markers are antiphospholipid antibodies–anticardiolipin antibodies, the lupus anticoagulant, or both'; APAS is characterized by the presence of circulating antiphospholipid antibodies–APA, in particular against cardiolipin–ACA, here used interchangeably, which overlap with lupus anticoagulants. Cf Lupus anticoagulant.

an·ti·phos·pho·lip·id an·ti·bod·y syn·drome

(APS, aPLS) (an'tē-fos-fō-lip'id an'ti-bod-ē sin'drōm)
A tendency for recurrent thrombosis together with recurrent abortion, thrombocytopenia, and neurologic disease, and elevated blood levels of antibodies against certain negatively charged phospholipids (e.g., cardiolipin, phosphatidylserine, and phosphatidylethanolamine).

antiphospholipid antibody syndrome

(ant″i-fos″fō-lip′ĭd) [ anti-phospholipid],

APAS

A condition characterized by hypercoagulability associated with high blood levels of IgG antibodies against phospholipids. Many affected patients have a systemic autoimmune disease, e.g., systemic lupus erythematosus, but others present only with a history of frequent arterial and venous thrombi or pregnancy loss. Recent evidence suggests that antiphospholipid antibodies play a role in approx. 20% of strokes, esp. in patients who do not have common risk factors for stroke. Antiphospholipid antibodies include lupus anticoagulant and anticardiolipins; the presence of the latter causes these patients to test positive for syphilis.

Thromboses caused by the syndrome are treated and prevented with heparin, warfarin, corticosteroids, or, in some instances, immunosuppressant drugs such as cyclophosphamide.

CAUTION!

Warfarin should not be used during pregnancy because of the risk of fetal malformations.
References in periodicals archive ?
Guidelines on the investigation and management of antiphospholipid syndrome.
Rituximab therapy for recurrent thromboembolic disease in antiphospholipid syndrome.
The prevalence of iron deficiency and anemia in the primary antiphospholipid syndrome patients and the controls.
In addition to cardiovascular disease, osteoporosis, Sjogren's syndrome, antiphospholipid syndrome, and autoimmune thyroid disease, other conditions that occur at an increased rate in patients with SLE include malignancies, rheumatoid arthritis, systemic sclerosis, myositis, vasculitis, autoimmune hepatitis, and infections.
Antiphospholipid syndrome is an autoimmune disorder of in vivo hypercoagulation and of importance to the cardiac anaesthetist; this subset of patients has a higher rate of perioperative thrombotic complications resulting in increased early and late morbidity and mortality following cardiac surgery (1).
Antibodies against PLs have been commonly found in patients with autoimmune diseases such as systemic lupus erythematosus and primary antiphospholipid syndrome, in which clinical manifestations (mainly thrombotic events) have been directly attributed to antibodies against PLs.
Some topics explored are apolipoproteins in primary antiphospholipid syndrome, the association of apolipoprotein E and delirium, apolipoproteins and lipids in an overweight man with familial hypertriglyceridemia, the structure of apoproteins of hen egg yolk low-density lipoproteins, and apoE for application in gene therapy.
In their October 1999 paper for Blood Coagulation & Fibrinolysis, David Berg and colleagues describe their blinded prospective study for testing the hypothesis that most people with CFS or fibromyalgia (FM) have antiphospholipid syndrome.
Reported causes of thrombophilia in HIV-infected subjects include antiphospholipid syndrome [1], increased platelet activation [1], elevated homocysteinemia [2], elevated plasma factor VII activity [2], lupus anticoagulant [3], activated protein C resistance [3], protein C deficiency [3,4], and acquired protein S deficiency [4-10].
Sam suffers from a rare condition called antiphospholipid syndrome, or Hughes syndrome, which has been linked to problems in pregnancy such as pre-eclampsia and miscarriages.