Introduction: Antiphospholipid syndrome (APS) is an autoimmune condition manifested by arterial and venous thrombosis, fetal loss, cardiac valvular damage, neurological white matter changes and renal dysfunction. APS is uncommon, with an incidence of 1-2/100,000; close to 80% are in women. We present a rare case of newly diagnosed APS in a 27 year old male. Description: A 27-year-old Hispanic male with no past medical history presented with a chief complaint of difficulty walking. Five weeks prior to admission he began to experience dry eyes and blurred vision, subjective fevers, night sweats, unintentional 20lbs weight loss, and progressive joint pain with inability to ambulate. Vital signs in the emergency room pertinent for temperature 38.5 ℃, blood pressure 126/81 mmHg, tachycardia 108 BPM, SpO2 100% on room air. Physical exam demonstrated tenderness to palpation of lower extremities. Laboratory analysis showed 2.2 x 103/uL white blood cells, hemoglobin 11.4 G/DL, and thrombocytopenia 144 x 103 /uL. Liver enzymes were elevated: ALT 58 U/L, AST 59 U/L. Creatinine was normal at 0.81 mg/dL, however the patient was noted to have an elevated erythrocyte sedimentation rate, 95 MM/HR, and C-reactive protein 47.7. Duplex ultrasound of the lower extremities bilaterally depicted diffuse deep venous thrombosis of right deep veins including the proximal femoral, popliteal, mid and distal femoral vein, and left distal femoral and popliteal vein, with mild residual flow. Given the concern for catastrophic antiphospholipid syndrome, the patient was started on full dose anticoagulation with intravenous heparin, high dose intravenous methylprednisolone, and plasmapheresis. Antinuclear antibody and rheumatoid factor were positive, and the anti-DNA DS antibody grossly elevated at 108 IU/mL. Discussion: Catastrophic Antiphospholipid Syndrome (CAPS) is a rare presentation of APS with mortality as high as 37%, requiring critical care monitoring given the high risk of decompensation. It is characterized by multiorgan dysfunction and thrombosis. A high index of clinical suspicion is essential to initiate prompt treatment. Our patient recovered well after plasmapheresis and was discharged home on apixaban.
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