Task force report on catastrophic antiphospholipid syndrome

Abstract

The catastrophic variant of the antiphospholipid syndrome (APS) is the most severe form of APS with acute multiple organ involvement and systemic inflammatory syndrome (SIRS). The hallmark of catastrophic APS (CAPS) is the histopathologic evidence of small vessel occlusions. At present, there are no studies on the pathophysiologic mechanisms of CAPS. A precipitating factor is reported in more than half of the patients. The first clinical manifestation at the time of the catastrophic episode is a pulmonary complication in 24% of the cases, a neurologic feature in 18% and a renal feature in 18%. Cerebral involvement is the most frequent cause of death followed by cardiac involvement, infection, and multiorgan failure. The first-line treatment should include the combination of anticoagulation against thrombosis plus glucocorticoids against the manifestations of SIRS plus plasma exchange and/or intravenous immunoglobulins to remove or block the antiphospholipid antibodies and the cytokines involved in the SIRS.