Antiphospholipid Antibody Syndrome

Wikis > Rheumatology > Antiphospholipid Antibody Syndrome

https://www.ncbi.nlm.nih.gov/pubmed/28644259?dopt=Abstract

Antiphospholipid Antibody Syndrome (aPL, APS) (Hughes syndrome; lupus anticoagulant; anticardiolipin antibody syndrome)

Antiphospholipid antibodies are directed against certain membrane phospholipids – these antibodies can be found in up to 2% of the normal population without symptoms. Results in recurrent vascular thrombosis, pregnancy loss and thrombocytopenia. Present in up to 50% of those with SLE and 30% of those with HIV.

Syndrome of CLOT – Clot, Livedo reticularis, Obstetrical loss, Thrombocytopenia

Antiphospholipid antibodies reduce the level of annexin V (potent anticoagulant)  increased clotting. One of more common causes of hypercoaguable states. Originally described in those with systemic lupus erythematosus – ‘primary aPL’ occurs in those without underlying disease.

Clinical features:
Arterial occlusion (gangrene, stroke, myocardial infarction); thrombosis (arterial and venous); venous occlusion; thrombocytopenia; livedo reticularis; recurrent foetal loss; neurologic abnormalities (chorea, multiple sclerosis like symptoms, epilepsy) valvular heart disease; lower limb ulcers (related to vascular complications and livedo reticularis).

Management:
Acute management of thrombosis.
Oral anticoagulants for prophylaxis, especially if pregnant.
Immunosuppressive do not prevent recurrent thrombosis, foetal loss or neurological symptoms.
Symptomatic management of complications.

http://www.jfas.org/article/S1067-2516(16)00003-X/abstract?rss=yes

 
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