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Anti-phospholipid antibody syndrome 

Anti-phospholipid antibody syndrome
Chapter:
Anti-phospholipid antibody syndrome
Source:
Oxford Textbook of Rheumatology (4 ed.)
Author(s):

Munther A. Khamashta

, Graham R. V. Hughes

, and Guillermo Ruiz-Irastorza

DOI:
10.1093/med/9780199642489.003.0120_update_002
Previous versions of this chapter are available. To view earlier versions of this chapter view the full site here.

The anti-phospholipid syndrome (APS) described almost 30 years ago, is now recognized as a major cause of deep vein thrombosis, stroke, and heart attacks in young people (<45 years of age). It is also the commonest treatable cause of recurrent miscarriages and a major cause of late fetal death. Other clinical manifestations are cardiac valvular disease, livedo reticularis, renal thrombotic microangiopathy, thrombocytopenia, haemolytic anaemia, epilepsy, and cognitive impairment. The presence of anti-phospholipid antibodies (aPL) has been closely related to the development of thrombosis and complications in pregnancy. However, not all patients with aPL will develop the clinical features. Lupus anticoagulant is generally thought to be more strongly associated with the risk of clinical manifestations of APS than anticardiolipin and anti ?2-glycoprotein I antibodies. The exact pathogenic mechanisms leading to thrombosis and/or pregnancy morbidity are poorly understood. Therapy of thrombosis is based on long-term oral anti-coagulation and patients with arterial events should be treated aggressively. Obstetric care is based on combined medical-obstetric high-risk management and treatment with aspirin and heparin.

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