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Semin Thromb Hemost 2008; 34(3): 256-266
DOI: 10.1055/s-0028-1082269
© Thieme Medical Publishers

Antiphospholipid Antibodies and the Antiphospholipid Syndrome: Clinical Significance and Treatment

Ronald A. Asherson1 [5] , Ricard Cervera2 , Joan T. Merrill3 , Doruk Erkan4
  • 1Professor of Immunology (Hon), Division of Immunology, The School of Pathology, University of the Witwatersrand; Consultant Rheumatologist, The Netcare Rosebank Hospital, Rosebank, Johannesburg, South Africa
  • 2Department of Autoimmune Diseases, Hospital Clinic, Barcelona, Catalonia, Spain
  • 3Clinical Pharmacology Program, Oklahoma Medical Research Foundation, RIA Section, Department of Medicine, University of Oklahoma, Oklahoma City, Oklahoma
  • 4Barbara Volcker Center for Women and Rheumatic Disease, Hospital for Special Surgery, Weill Medical College of Cornell University, New York, New York
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Publication History

Publication Date:
21 August 2008 (online)

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ABSTRACT

This article provides a review of the various types of antiphospholipid (aPL) antibodies and antiphospholipid syndromes, their prevalence, presumed origin, relationship to autoimmunity in general, and their role in the body's defenses and apoptosis. New hypotheses such as the role of antibodies to β2 glycoprotein I (β2GPI) and the signaling of toll-like receptors are also discussed, as is the spectrum of clinical manifestations associated with the demonstration of these antibodies, now assumed to be “pathogenic.” A distinction is made between antibodies present in sera of patients with a variety of microangiopathic syndromes (MAPS; e.g., HELLP syndrome, thrombotic thrombocytopenic purpura, and thrombotic microangiopathic syndromes). In these conditions, the antibodies might not be “pathogenic” but, alternatively, generated by small vessel endothelial damage. These conditions are differentially referred to as microangiopathic antiphospholipid–associated syndromes, and they should be differentiated from the microvascular occlusions that are seen in the antiphospholipid syndrome. Current treatments of the antiphospholipid syndrome are briefly reviewed.

KEYWORDS

Antiphospholipid (aPL) antibodies - antiphospholipid syndromes - APS - treatment

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5 deceased

Ricard CerveraM.D. 

Servei de Malalties Autoimmunes, Hospital Clinic

Villarroel 170, 08036 Barcelona, Catalonia, Spain

Email: rcervera@clinic.ub.es