Current insight into diagnostics and pathophysiology of the antiphospolipid syndrome

The diagnosis of the antiphospholipid syndrome, a non-inflammatory autoimmune disease characterized by thrombosis or pregnancy morbidity in the presence of antiphospholipid antibodies, depends greatly upon laboratory diagnostics. The diagnostic value of all available assays to detect antiphospholipid antibodies and the anticardiolipin assay in particular, is a matter of ongoing debate. Although the presence of lupus anticoagulant correlates best with thrombosis, accurate determination is not always possible due to anticoagulant treatment. Data on the predictive value of alternatives such as the anti-β2-glycoprotein I and the anti-prothrombin antibody assay are insufficient and prospective cohort studies are needed. Determining antiphospholipid antibody profiles seems to increase diagnostic specificity. Substantial progress has been made in unravelling the pathophysiological mechanisms underlying the antiphospholipid syndrome. Several cellular receptors for antibody-β2-glycoprotein I complexes have been identified and their roles in cellular activation are being investigated. In vivo data should provide more insight into the importance of the interaction with individual receptors.