Abstract
The catastrophic variant of the antiphospholipid syndrome (APS) is the most severe form of APS with acute multiple organ involvement and small vessel thrombosis. At present, there are no studies on the pathophysiological mechanisms of catastrophic APS. The two theoretical explanations for the clinical manifestations of catastrophic APS are the development of thrombosis and the systemic inflammatory response syndrome (SIRS). From retrospective study data, first-line therapies should always include the combination of anticoagulation against thrombosis plus glucocorticoids against manifestations of SIRS plus plasma exchange and/or intravenous immunoglobulins to remove or block the antiphospholipid antibodies and cytokines involved in the SIRS. This review is focused on current management of catastrophic APS and some of the potential new therapeutic approaches.
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