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New targeted therapies for treatment of thrombosis in antiphospholipid syndrome

Published online by Cambridge University Press:  13 November 2007

Silvia S. Pierangeli*
Affiliation:
Division of Rheumatology, Department of Internal Medicine, University of Texas Medical Branch, Galveston, TX, USA.
Mariano E. Vega-Ostertag
Affiliation:
Instituto Fares Taie, Mar del Plata, Buenos Aires, Argentina.
Emilio B. González
Affiliation:
Division of Rheumatology, Department of Internal Medicine, University of Texas Medical Branch, Galveston, TX, USA.
*
*Corresponding author: Silvia S. Pierangeli, Division of Rheumatology, Department of Internal Medicine, 301University Boulevard, Galveston, TX 77555-1165, USA. Tel: +1 409 772 0222; Fax: +1 409 772 0223; E-mail: sspieran@utmb.edu

Abstract

Antiphospholipid (aPL) antibodies (Abs) are associated with thrombosis and pregnancy loss in antiphospholipid syndrome (APS), a disorder initially characterised in patients with systemic lupus erythematosus (SLE) but now known to occur in the absence of other autoimmune disease. There is strong evidence that aPL Abs are pathogenic in vivo, from studies of animal models of thrombosis, endothelial cell activation and pregnancy loss. In recent years, progress has been made in characterising the molecular basis of this pathogenicity, which includes direct effects on platelets, endothelial cells and monocytes as well as activation of complement. This review summarises the clinical manifestations of APS and current modalities of treatment, and explains recent advances in understanding the molecular events triggered by aPL Abs on target cells in coagulation pathways as well as effects of aPL Abs on complement activation. Based on this information and on additional scientific evidence using in vitro and in vivo models, new potential targeted therapies for treatment and/or prevention of thrombosis in APS are proposed and discussed.

Type
Review Article
Copyright
Copyright © Cambridge University Press 2007

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References

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Further reading, resources and contacts

The eMedicine entries for antiphospholipid syndrome (APS) by Elise Belilos and by Barry L. Myones are updated practical guides for physicians that include relevant information on clinical manifestations and current treatment in APS, a brief synopsis on history and pathophysiology, and updated classification criteria for definite APS: