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Extensive investigation of antiphospholipid syndrome (APS) has improved our knowledge of the disease, but raised new questions. Although the pathogenic role of antiphospholipid antibodies (aPL) is widely accepted, the fact that aPL induces thrombotic events only occasionally suggests the need for a “second hit” to display the thrombogenic effect. Infectious agents are thought to trigger autoantibody production through a molecular mimicry mechanism, but may also induce an inflammatory process that eventually favours clotting. The involvement of complement and cytokines in the pathogenesis further supports the role of inflammation in APS as well as the possibility for new therapeutic approaches. The question of whether other environmental triggers or a genetic individual susceptibility can behave as a second hit is still open. The clinical involvement of different organs and systems poses the question of whether the syndrome should be considered a true systemic autoimmune disease, rather than an acquired autoimmune coagulopathy. A positivity for more than one laboratory test displays a higher predictive value for clinical events, but it is still not known which other parameters are useful in clinical practice to identify all the aPL positive subjects at risk for manifestation. In particular, there is no clear evidence when a more aggressive treatment must be used in primary prophylaxis. The standard anticoagulant therapy has dramatically changed the prognosis of APS; however, the vascular and/or obstetrical recurrences in spite of the treatment pose the question whether additional therapeutic strategies (ie, immunosuppression) should be used, or whether alternative therapies (ie, inhibitory/tolerogenic peptides) should be sought.
Antiphospholipid syndrome (APS) was first described in 1983.1 Extensive investigations and several congresses have addressed the basic and the clinical aspects of APS.2–8 Consequntly, 25 years later a large knowledge base has been built up on the aetiology, the pathogenesis and the therapy of …
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