CATASTROPHIC ANTIPHOSPHOLIPID SYNDROME: A RARE BUT SERIOUS CONDITION

Abstract

SESSION TITLE: Medical Student/Resident Critical Care Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Catastrophic Antiphospholipid Syndrome (CAPS) is a rare, life threatening complication of Antiphospholipid Syndrome (APS). Fewer than 1% of individuals with APS develop CAPS over their lifetime; but the mortality of this condition is greater than 50%. Due to the aggressive nature of this disease, early diagnosis and management is imperative for potential survival. Here we describe a case of probable CAPS with a favorable outcome in a critically ill patient. CASE PRESENTATION: A 60 year old woman with systemic lupus erythematosus (SLE) and associated lupus nephritis on hemodialysis, APS with anticardiolipin antibodies, paroxysmal atrial fibrillation on anticoagulation with warfarin presented with abdominal pain. She was on immunosuppressive therapy with belimumab and prednisone for about a year. An ultrasound and a CT scan of the abdomen were concerning for acute acalculous cholecystitis. She was deemed to be high risk for cholecystectomy and was conservatively managed with intravenous antibiotics with a plan for non emergent cholecystostomy tube insertion. Her hospital course was complicated by Clostridium perfringens bacteremia and worsening encephalopathy, which was initially thought to be toxic-metabolic secondary to sepsis and uremia. The patient was transferred to the MICU and had extensive work up including MRI brain, Lumbar Puncture and EEG which were all unrevealing. Her clinical picture was not consistent with PRES, lupus cerebritis or HUS/TTP. Anticoagulation was switched to from Coumadin to IV heparin. She was subsequently intubated due to her worsening mental status, but there was difficulty in weaning her off the ventilator as she remained obtunded despite holding all sedatives and correction of metabolic abnormalities. At this time, we repeated an EEG which showed diffuse encephalopathy with no epileptiform activity. A repeat MRI of the brain showed acute infarcts in the anterior and posterior cerebral artery areas of bilateral cerebral hemispheres (new compared to the MRI 5 days ago) despite being on therapeutic anti-coagulation. Given these clinical and imaging findings, there was high clinical suspicion for CAPS. After discussion with rheumatology and hematology services, the patient was started on plasma exchange, high dose steroids and IVIg. A TEE was done to rule out cardio-embolic source of CVA and did not show any vegetation or thrombus. There was dramatic improvement after this triple therapy and she was successfully extubated and transferred out of the ICU DISCUSSION: CAPS is an autoimmune disease characterized by disseminated intravascular thrombosis resulting in multi-organ failure over a short period (1 week) and is associated with high morbidity and mortality. CONCLUSIONS: An aggressive multidisciplinary treatment strategy is indicated including early diagnosis and treatment with triple therapy (plasmapheresis, IVIG and steroids) for positive outcomes. Reference #1: Aguiar CL, Erkan D. Catastrophic antiphospholipid syndrome: how to diagnose a rare but highly fatal disease. Ther Adv Musculoskelet Dis. 2013;5(6):305-314. doi:10.1177/1759720X13502919 DISCLOSURES: No relevant relationships by Neha Bhandari, source=Web Response No relevant relationships by Avantika Nathani, source=Web Response