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.
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).
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.