Case Report 36-Year-Old Female with Catastrophic Antiphospholipid Syndrome Treated with Eculizumab: A Case Report and Review of Literature

Catastrophic antiphospholipid syndrome (CAPS) is a rare but potentially life-threatening condition characterized by diffuse vascular thrombosis, leading to multiple organ failure developing over a short period of time in the presence of positive antiphospholipid antibodies (aPL). CAPS is a severe form of antiphospholipid syndrome, developing in about 1% of cases of classic antiphospholipid syndrome, manifesting as microangiopathy, affecting small vessels of multiple organs. It is acute in onset, with majority of cases developing thrombocytopenia and less frequently hemolytic anemia and disseminated intravascular coagulation. Lupus anticoagulant and anticardiolipin antibodies have been reported as predominant antibodies associated with CAPS. Treatment options often utilized in CAPS include anticoagulation, steroids, plasma exchange, cyclophosphamide therapy, and intravenous immunoglobulin therapy. Even though the reported incidence of this condition is considered to be low, the mortality rate is approaching 50%. The high rate of mortality should warrant greater awareness among clinicians for timely diagnosis and treatment of this life-threatening condition. Studies have shown that complement activation plays a key role in the pathogenesis of aPL mediated thrombosis in CAPS. We report a case of a 36-year-old female admitted with clinical and laboratory findings consistent with CAPS successfully treated with eculizumab, a terminal complement inhibitor. Case Presentation We would like to present a case of a 36-year-old female who was admitted to our hospital after being found in the bathtub with decreased responsiveness and profound weakness. The patient had no medical problems and had been in her usual state of health until one month before admission when she developed frequent headaches and blurry vision. Per family the patient had become more withdrawn recently and was not allowing anyone to visit her home. On examination in the Emergency Department, the patient was found to be hypertensive, to have blood pressure of 207/148 mmHg, to have heart rate of 110 beats per minute, and appeared confused and dehydrated, with multiple bruises over her body. Laboratory examination revealed white blood cell (WBC) count of 20.5/nL, hemoglobin of 12.3 g/dL, and platelets of 44,000/ L. Patient's renal function was altered, presenting anion gap metabolic acidosis (suspected starvation ketosis) and respiratory alkalosis. Creatinine on admission was noted to be 7.0 mg/dL. Prothrombin time (PT) was 11.6 sec (reference range: 9.5-12.2 sec), activated partial thromboplastin time (aPTT) was 21.5 sec (reference range: 20.1-31.2 sec), and fibrinogen was 345 mg/dL. Mixing study was not performed at the time, since coagulation panel did not show prolongation of PTT. Troponins were elevated with no ST segment changes on electrocardiography compatible with non-ST segment myocardial infarction (NSTEMI). Ophthalmology was consulted and stated that visual loss and color blindness was likely secondary to intraretinal hemorrhage. A computed tomography (CT) of the brain was performed, whic