Catastrophic Antiphospholipid Syndrome

Catastrophic antiphospholipid syndrome (CAPS) is a rare, potentially lethal, multiple-faceted, systemic disease, often triggered by a precipitating factor. Its tissue lesions are mainly the consequence of an antiphospholipid antibody (aPL Abs)-mediated thrombotic microangiopathy associated with a so-called cytokine storm. Bleeding may coexist, making it difficult to manage. In an emergency situation, the differential diagnosis is not always straightforward in patients not known to be aPL Abs carriers, or with no history of thrombotic or obstetric complications. What we have gradually learned about its clinical presentation and therapeutic management mainly comes from the CAPS registry. So far, there have been no randomized controlled trials to guide treatment. The current therapeutic recommendations insist on prescribing first-line triple therapy, combining early anticoagulation, immunosuppression, and the removal/neutralization of aPL Abs, as early as possible. The prognosis has improved but remains bleak. Future research to develop pathophysiological treatments blocking the activation of target cells by aPL Abs is warranted.