Pathophysiology of the antiphospholipid syndrome.
Antiphospholipid syndrome is a distinct disorder with the clinical features of recurrent thrombosis in the venous or arterial circulation and fetal losses. Its serological marker is the presence of antiphospholipid antibodies in the blood of these patients. The relation between the presence of antibodies against anionic phospholipids and thromboembolic complications is well established over the last 25 years but the pathophysiology of the syndrome is largely unclear. Even after all these years, there is a persisting debate about the specificity and sensitivity of the assays for the detection of antiphospholipid antibodies. We now accept that antibodies to beta2-glycoprotein I rather than to anionic phospholipids are the major pathological antibodies, although there is no clear consensus on how the presence of these antibodies correlates with the different clinical manifestations of the syndrome. In this review, we discuss the current methods of detection of the antibodies and our insight into the pathobiology of the syndrome. We propose a mechanism for describing how the presence of anti-beta2-glycoprotein I antibodies relates to the different clinical manifestations observed.
de Groot
P G
PG
Department of Haematology, University Medical Center Utrecht, Utrecht, The Netherlands. ph.g.degroot@azu.nl
Derksen
R H W M
RH
D017152:Antibodies, Antiphospholipid / Q000097:blood*; D016736:Antiphospholipid Syndrome / Q000175:diagnosis* / Q000276:immunology* / Q000473:pathology; D001792:Blood Platelets / Q000378:metabolism; D018360:Crystallography, X-Ray; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006023:Glycoproteins / Q000276:immunology; D006801:Humans; D020935:MAP Kinase Signaling System; D008954:Models, Biological; D008958:Models, Molecular; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000175:diagnosis / Q000473:pathology; D053482:beta 2-Glycoprotein I
D017152:Antibodies, Antiphospholipid; D006023:Glycoproteins; D053482:beta 2-Glycoprotein I