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Antiphospholipid Antibody Syndrome

  • Tadej AvčinEmail author
Reference work entry

Definition/Classification

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).

The classification criteria for APS were developed by consensus and designate patients who suffered from vascular thrombosis or pregnancy morbidity associated with the presence of aPL, detected on two or more occasions at least 12 weeks apart ( Table 160.1). These criteria were developed for classification of adult patients...

Keywords

Systemic Lupus Erythematosus Thrombotic Event Lupus Anticoagulant Livedo Reticularis Pregnancy Morbidity 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Supplementary material

Video 160.1

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.

AVI file: 24810 kB

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Copyright information

© Springer-Verlag Berlin Heidelberg 2012

Authors and Affiliations

  1. 1.Department of Allergology, Rheumatology and Clinical ImmunologyUniversity Children’s Hospital Ljubljana, University Medical CenterLjubljanaSlovenia

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