“…14 Figure 1 summarizes coagulation mechanisms and targets for actions of different anticoagulants. 15 Anticoagulation, in combination with corticosteroids, plasma exchange, and/or intravenous immunoglobulin (IVIG), is standard treatment for CAPS. 3,4,16 Non-criteria APS manifestations, which include livedo reticularis, thrombocytopenia, hemolytic anemia, cardiac valve disease, nephropathy, skin ulcers, and cognitive dysfunction, 1 are generally anticoagulant-refractory and require consideration of further therapeutic approaches.…”