Encyclopedia of Heart Diseases

2011 Edition
| Editors: M. Gabriel Khan

Antiphospholipid Antibody Syndrome

Reference work entry
DOI: https://doi.org/10.1007/978-1-60761-219-3_15


The antiphospholipid syndrome (APS) is an autoimmune thrombophilic condition that is marked by the presence of antibodies that recognize phospholipid-binding proteins (Rand 2007).

The pathophysiologic process associated with antiphospholipid antibody syndrome remains uncertain; however, it includes inhibition of prostacyclin formation or protein C activation. Antibodies affect  platelets, limit the production of endothelium-derived relaxing factor, and possibly inhibit the prekallikrein-mediated intrinsic pathway of fibrinolysis. Antiphospholipid antibodies are associated with recurrent fetal loss (typically second- or third-trimester miscarriages),  thrombocytopenia, false-positive Venereal Disease Research Laboratory test results, painful leg ulcerations, cardiac valve alterations, and livedo reticularis (Roach et al. 2008).

The principal autoantigen of antiphospholipid antibody syndrome has been shown to be beta-2-glycoprotein 1 (apolipoprotein H), a protein that binds...

This is a preview of subscription content, log in to check access.



  1. Lanthier S, Kirkham FJ, Mitchell LG et al (2004) Increased anticardiolipin antibody IgG titers do not predict recurrent stroke or TIA in children. Neurology 62:194–200PubMedGoogle Scholar
  2. Lockshin MD, Erkan D (2003) Treatment of the antiphospholipid syndrome. N Engl J Med 349:1177–1179PubMedGoogle Scholar
  3. Rand JH (2007) The antiphospholipid syndrome. Hematology 2007(1):136–142Google Scholar
  4. Roach ES, Golomb MR, Adams R et al (2008) Management of stroke in infants and children: a scientific statement from a Special Writing Group of the American Heart Association Stroke Council and the Council on Cardiovascular Disease in the Young. Stroke 39(9):2644–2691PubMedGoogle Scholar

Suggested Reading

  1. Cervera R, Khamashta MA, Shoenfeld Y et al (2009) Morbidity and mortality in the antiphospholipid syndrome during a 5-year period: a multicentre prospective study of 1000 patients. Ann Rheum Dis 68(9):1428–1432PubMedGoogle Scholar
  2. Giannakopoulos B, Krilis SA (2009) How I treat the antiphospholipid syndrome. Blood 114:2020–2030PubMedGoogle Scholar
  3. Giannakopoulos B, Passam F, Rahgozar S et al (2007) Current concepts on the pathogenesis of the antiphospholipid syndrome. Blood 109:422–430PubMedGoogle Scholar
  4. Tarr T, Lakos G, Bhattoa HP et al (2007) Analysis of risk factors for the development of thrombotic complications in antiphospholipid antibody positive lupus patients. Lupus 16:39–45PubMedGoogle Scholar

Copyright information

© Springer Science+Business Media LLC 2011