CASE PRESENTATION: A 45-year-old female with a history of systemic lupus erythematosus and end-stage renal disease presented to an outside hospital with intractable vomiting. Computed tomography of the abdomen showed fluid collection in the left upper quadrant and partial thrombosis of the superior mesenteric vein (SMV). She then developed polymicrobial Gram-negative bacteremia (Proteus vulgaris, Klebsiella pneumonia) as well as thrombocytopenia, lower GI bleeding, and respiratory failure. She was transferred to our facility. On arrival, she was suspected to have CAPS with thrombotic storm, based on the presence of digital ischemia, right radial artery and SMV thrombus. She received intravenous immunoglobulin (IVIg) and steroids but did not tolerate anticoagulation due to GI bleeding. Blood and peritoneal fluid cultures grew Enterococcus faecium. Due to increasing vasopressor requirements and intra-abdominal pressures she was taken emergently for surgical washout to remove abundant intra-abdominal pus. Biopsies of black lesions on the colon revealed thrombosis of the microvasculature and necrosis. Beta-2 glycoprotein IgA was 52 GPI IGA units, and RNP Ab was 1.1 AI. Despite multiple washouts, steroids, IVIg and plasmapheresis, she died on day 21 of hospitalization.