Case Presentation: 64 years old African American smoker menopausal female on HRT with PMH of Hypertension presented to ED with sudden onset blurry vision in both eyes of one day duration and generalized malaise for one week. Patient noted to have Left sided weakness in ED. No history of headache, change in speech, numbness or weakness in other extremities Patient had low grade fever as well. CT head revealed acute multiple infarcts of both lower occipital, right parietal and frontal lobes. EKG with ST elevation in leads II, III and aVF with elevated troponin. Labs were significant for leukocytosis, thrombocytopenia with platelets of 137, mildly elevated INR and AKI with Cr of 1.17. Emergent left heart catheterization was negative for occlusive coronaries disease. The patient received treatment for stroke and started on broad spectrum antibiotics given concern for Endocarditis as presumed initial Diagnosis in setting of possible cardioembolic etiology of stroke along with fever and elevated WBC. Transesophageal Echo showed Vegetations on the aortic valve with severe aortic regurgitation. Multiple surveillance blood cultures were negative. Subsequent labs revealed severe anemia with worsening thrombocytopenia with platelets of 87 and 46 and positive DIC panel with LDH of 1000. peripheral smear with few schistocytes. Venous Doppler revealed acute DVT in bilateral lower Ext., Right cephalic and Left brachial vein thrombosis. Hematology and Rheumatology team were consulted and concern was for possible catastrophic Antiphospholipid syndrome in light of disseminating thrombosis involving multiple organs developing within few days. Full Rheumatology work up was negative results except for aPL, ACA IgM High Positive and LA Positive, B-2 GP1 was negative. The patient was diagnosed with probable catastrophic APS and started on high dose IV steroids along with Heparin IV infusion with caution given recent extensive stroke. Next day, the patient developed acute respiratory distress with subsequent PEA cardiac arrest and passed away. Autopsy showed multiple fibrin vegetations attached to aortic valve and histopathology revealed multiple areas of intravascular thrombosis and infarcts involving Right and Left Ventricles/Brain/Spleen and Kidneys which supported the diagnosis of CAPS
Discussion: Antiphospholipid syndrome (APS) is an uncommon condition characterized by arterial and venous thrombosis and/or pregnancy morbidity with presence of antiphospholipid antibodies. Catastrophic APS is a rare life threatening disease that develops in 1% of patients with APS. It represents accelerated form of APS in which patients present with catastrophic disseminated thrombotic events involving multiple organs resulting in multiorgan failure within a week. Its more common in female with mean age of presentation of thirty-eight. Our patient developed fatal Catastrophic APS at the age of sixty four as the initial presentation of APS and was diagnosed with probable catastrophic APS as she met three out of four of CAPS diagnostic criteria. Diagnosis of this syndrome might be difficult given the fact that in most of the cases, the patients initial clinical presentation can be explained by other common medical conditions
Conclusions: Catastrophic APS is rare hence a fatal disease with mortality rate of 40% without treatment. Physicians should have high suspicion of this disease in patients who presenting with multiple thrombotic events within a short period of time in an effort of early diagnosis and treatment of this lethal disease