Antiphospholipid syndrome

Abstract. The antiphospholipid syndrome (APS) was reported in theearly 1980s as the association of thrombosis, recurrentpregnancy loss in the presence of anticardiolipin antibodies(aCL) and/or lupus anticoagulant (LA). Since then, many otherclinical manifestations have been associated with aPL. Almostany organ and tissue may be involved in the disease, includingthe brain, the heart, the kidneys, the placenta and many more.aPL are a heterogeneous group of autoantibodies that aredetected by immunoassays and functional coagulation tests. Theantigenic targets are negatively charged phospholipids and serumphospholipid-binding proteins. Despite the strong associationbetween aPL and thrombosis, the pathogenic role of aPL in thedevelopment of thrombosis has not been fully elucidated.Proposed mechanisms include antibody-mediated interference withcoagulation homeostasis, activation of platelets and endothelialcells and a T-cell immune response to serum phospholipid-bindingproteins. The mainstay of therapy is anticoagulation, whereasimmunosuppression seems to be ineffective. Recommendations forthe management of thrombosis in the antiphospholipid antibodysyndrome have been based largely on retrospective case series.Several prospective clinical trials are currently underway andtheir results will probably lead to a more precise therapeuticapproach of this problem.