Antiphospholipid Antibody Syndrome
The antiphospholipid antibody syndrome (APS) is a multisystemic autoimmune disease characterized by thromboembolic events, pregnancy morbidity, hematologic, dermatologic, neurologic, and other manifestations in the presence of elevated titers of antiphospholipid antibodies (aPL). Antiphospholipid antibodies are a heterogeneous group of autoantibodies directed against negatively charged phospholipids or phospholipid-binding plasma proteins. The most relevant aPL for identifying patients at risk for immune-mediated thrombosis are anticardiolipin antibodies (aCL), antibodies against β2 glycoprotein I (anti-β2GPI) and lupus anticoagulant (LA).
KeywordsSystemic Lupus Erythematosus Thrombotic Event Lupus Anticoagulant Livedo Reticularis Pregnancy Morbidity
Choreoathetotic movement disorder associated with positive antiphospholipid antibodies in a teenage girl with SLE. The patient showed remarkable improvement after combination therapy with pulse methylprednisolone, intravenous gammaglobulin, and pulse cyclophosphamide.
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