Antiphospholipid syndrome (APS) is an autoimmune clinic-pathologic disorder characterized by thrombosis and/or pregnancy morbidity along with persistent antiphospholipid (aPL) antibodies. Patients with APS can experience venous, arterial, and/or microvascular thrombosis. Catastrophic antiphospholipid syndrome (CAPS) is an unusual but often fatal complication of APS distinguished by microvascular thrombosis manifesting as multiorgan failure in three or more organs. Severity correlates with extent of thrombosis and the organs involved, and the damage occurring within one week, or up to one month after initial onset. Catastrophic Antiphospholipid Syndrome (CAPS) is a life-threatening complication of APS requiring complex management to optimize patient outcome. 

We describe a 40-year-old woman with APS with history of biliteral leg muscle vein thrombosis, high D-dimer level and slightly thrombocytopenia.She developed sudden onset of pulmonary arteria thrombosis companied with a transient loss of consciousness without obvious predisposing factors accompanied by profuse sweating, nausea, and retching, lasting for more than ten seconds, along with a decrease in oxygen saturation while on warfarin with a therapeutic INR and inferior vena cava filter. Despite unfractionated heparin combine with aspirin and initial clinical improvement, high D-dimer level developed requiring high dose of methylprednisolone, immunoglobulin. Hospitalization was complicated further by thrombosis of the painful and swollen on his biliteral leg muscle.

Although the inciting event for her thrombotic storm remains uncertain, the level of C5b-9 decreased, anti-complement therapy with eculizumab provided rapid and durable lesion resolution. Eculizumab was discontinued after 6 months and patient remains in remission without recurrent thrombosis. This case provides insight on the management of CAPS, including the use of eculizumab. She developed sudden left leg muscle swollen and pain and unable walking. She experienced a transient loss of consciousness without obvious predisposing factors accompanied by profuse sweating, nausea, and retching, lasting for more than ten seconds, along with a decrease in oxygen saturation. Pulmonary artery CTA indicated pulmonary embolism, and anticoagulant therapy was administered. high D-dimer level (72.96 ug/mL), She was administered with intravenous immunoglobulin. Although the inciting event for her thrombotic storm remains uncertain, anti-complement therapy with eculizumab provided rapid and durable lesion resolution. Eculizumab was discontinued after 6 months and patient remains in remission without recurrent thrombosis. This case provides insight on the management of CAPS, including the use of eculizumab.

This case provides insight on the management of CAPS, including the use of eculizumab.