The anti-phospholipid antibody syndrome: clinical and serological aspects

The association of thromboses and/or cytopenias with anti-phospholipid antibodies (aPL), the anti-phospholipid syndrome (APS), is well recognized. The syndrome may be primary or occur within systemic lupus erythematosus (SLE). The notion of the syndrome occurring within SLE is important since patients found to have aPL may be at risk for developing APS manifestations, those who develop some manifestations may be at risk for developing others and, finally, SLE patients with this syndrome may need special treatment. There are subtle differences between the primary and the secondary forms, mostly due to the frequently higher and more persistent autoantibody levels in the primary and the influence of lupus in the secondary. These syndromes may be related to various antigen/antibody systems in which phospholipids participate either directly or through their effect on the proteins that bind them. Similar clinical manifestations also occur in patients who have serum antibodies to such proteins (e.g. β2-glycoprotein-I) in the absence of phospholipid. Some of these antibodies may even be more important pathogenically than the antibodies against cardiolipin that were originally described. Testing for the latter is, however, still the first choice when suspecting an anti-phospholipid syndrome. If this is negative in this situation, a search for the other autoantibodies is indicated.