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