A case of catastrophic antiphospholipid antibody syndrome.

Catastrophic antiphospholipid antibody syndrome (CAPS) is a rare and severe variant of antiphospholipid antibody syndrome (APS) characterized by wide-spread small vessel thrombosis, multi-organ failure and positive antiphospholipid antibodies. CASE PRESENTATION: A 25 year old Caucasian male was admitted to the hospital with acute pulmonary edema, acute kidney injury and anemia. Initial laboratory results showed microcytic anemia with thrombocytopenia. Transthoracic echocardiography revealed hypokinetic left ventricle with EF 30%.Two days later he was started on hemodialysis. Head CT, lumbar puncture, WBC with differential and bone marrow biopsy were all normal. Due to persistent agitation and fever on day 5, he underwent MRI brain which showed numerous sub-acute infarcts in bilateral frontal, parietal, occipital and temporal lobes. A venous Doppler of the lower extremities showed bilateral deep venous thromboses. Immunology workup revealed a positive ANA at 1:180, as well as positive lupus anticoagulants. Patient was started on plasmapheresis, steroids, and anticoagulation with marked clinical improvement. DISCUSSION: The diagnostic criteria for CAPS (1) includes multisystem organ involvement with microthromboses in the setting of a positive antiphospholipid antibody occurring within a week of the acute presentation. This syndrome is reported to be seen in less than 1% of patients with APS and is rarely preenting as an initial manifestation of the disease. The mortality rate in the CAPS registry is 44% with cerebral involvement being the main cause of death in 27% of the cases. The highest recovery rate was observed in patients who received combination of anticoagulants, corticosteroids and plasma exchange. The CAPS registry currently has more than 350 cases worldwide including our case. New agents including rituximab and eculizumab warrant further study as options of treatment in refractory cases. CONCLUSIONS: CAPS is a medical emergency which requires early identification and aggressive management to improve outcomes. Clinicians need a heightened awareness of this disease state when managing patients with acute thrombosis of multiple organs with no clear etiology