Pathogenic mechanisms mediating antiphospholipid syndrome.
Antiphospholipid antibodies are the marker for antiphospholipid syndrome. There is evidence that these autoantibodies lead to both thrombotic diathesis and obstetrical manifestations. Besides the known interaction with soluble coagulation factors, in vitro and in vivo experimental models and studies in humans recently have shown the ability of antiphospholipid antibodies to modulate functions of cells involved in coagulation homeostasis. These findings support a new hypothesis to explain the paradox of the prolongation of coagulation assays in vitro and the association with thrombophilic diathesis in vivo. Obstetrical manifestations have been linked to a direct antibody effect on the trophoblast leading to defective placentation that is not necessarily associated with thrombotic phenomena. Phospholipid binding proteins such as beta 2 -glycoprotein I appear to behave as a bridge between circulating antiphospholipid antibodies and cellular targets.
Meroni
P L
PL
Allergy and Clinical Immunology Unit, IRCCS Istituto Auxologico Italiano, Department of Internal Medicine, University of Milan, Italy. pierluigi.meroni@unimi.it
Riboldi
P
P
D000818:Animals; D017152:Antibodies, Antiphospholipid / Q000502:physiology*; D016736:Antiphospholipid Syndrome / Q000097:blood / Q000209:etiology*; D004195:Disease Models, Animal; D005260:Female; D005313:Fetal Death / Q000209:etiology / Q000503:physiopathology; D006801:Humans; D066298:In Vitro Techniques; D051379:Mice; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000209:etiology / Q000503:physiopathology; D013927:Thrombosis / Q000503:physiopathology
D017152:Antibodies, Antiphospholipid