Case Presentation: A 30-year-old woman with triple positive (Beta-2-glycoprotein IgG/IgM, anticardiolipin IgG/IgM, and lupus anticoagulant) antiphospholipid syndrome (APS) taking apixaban, aspirin, and hydroxychloroquine presented to the emergency department for progressive confusion, emesis, and hematochezia found to be in cardiogenic shock due to right ventricular failure with critical limb ischemia due to complete right popliteal artery occlusion. She was promptly intubated; started on IV heparin and vasopressors; and went to the operating room for placement on VA ECMO and completion of right popliteal balloon angioplasty, thrombectomy, and 4 compartment fasciotomy without return of dopplerable pulses. She was found to have right deep venous thrombi in the right common femoral vein and saphenous veins, as well as nonbacterial thrombotic endocarditis. Based on multiple thromboses with the history of triple positive APS, Catastrophic APS was suspected. She was started on pulse dose corticosteroids and plasmapheresis. Despite early, aggressive treatment for CAPS, the patient was unresponsive after sedation weaning. CT angiogram of the head demonstrated occlusion of the distal right middle cerebral artery (MCA) and proximal left MCA, with complete territorial infarct on the right and large left MCA infarct. The patient’s family made the decision to withdraw care.
Discussion: This case illustrates the intricacies of APS medication management in a patient who ultimately died from CAPS. Warfarin is the first line anticoagulant for people with APS, as it decreases the rate of thrombotic events more than direct oral anticoagulants (DOACs), like apixaban. However, DOACs have been recommended for people with APS who have lower-risk disease or who cannot tolerate warfarin. In addition, the patient was taking aspirin, which reduces the risk of first arterial thrombotic events and possibly of recurrent thromboses. Despite the second-line anticoagulation choice, the patient had been taking hydroxychloroquine, which reduces antiphospholipid antibody titers, decreasing the risk for CAPS. Therefore, this is a unique case of APS managed on an alternative regimen – apixaban, aspirin, and hydroxychloroquine – which resulted in fatality due to the CAPS manifestation of APS which affected the right lower extremity, mitral valve, and bilateral MCAs.
Conclusions: The CAPS disease process remains highly fatal – despite inpatient treatment with steroids, plasmapheresis, and anticoagulation – as in our case. Outpatient, risks and benefits must be considered before treating APS with a DOAC as opposed to warfarin, the first line treatment, as adequate anticoagulation is crucial in preventing life-threatening complications, such as CAPS. Optimal medical management of APS is critical in reducing the risk of CAPS, and early recognition of CAPS is crucial for aggressive treatment.