A life-threatening “thrombotic storm” of multi-organ micro & macro thrombosis in patients with antiphospholipid syndrome (known or unknown).
Triggered circulating antibodies (usually by infection, but can be prompted by malignancy, pregnancy, and lupus itself) cause endothelial disruption and inflammation leading to prothrombotic state, commonly with SIRS response.
Mortality is high at an estimated 40%.
Confirm diagnosis with antiphospholipid antibody titers.
Treat ASAP with unfractionated heparin, corticosteroids, and Hematology consultation for plasma exchange and/or IVIG.
Cervera R, Rodriguez-Pinto I, Colafrancesco S, et al. 14th International Congress on Antiphospholipid Antibodies Task Force. Report on catastrophic antiphospholipid syndrome. Autoimmun Rev 2014; 13:699–707.
