FATAL CATASTROPHIC ANTIPHOSPHOLIPID SYNDROME WITH THROMBOTIC STORM IN A PATIENT WITH TRIPLE POSITIVE APS

SESSION TITLE: Critical Care Case Report Posters 18 SESSION TYPE: Case Report Posters PRESENTED ON: 10/09/2023 02:10 pm - 02:55 pm INTRODUCTION: Catastrophic antiphospholipid syndrome (CAPS) is a rare, but life-threatening form of antiphospholipid syndrome (APS) that can occur after infection, recent surgery, or medication non-compliance in patients who are predisposed to complement dysregulation. It is characterized by severe thrombotic complications, usually microvascular as well as large vessel thrombosis affecting multiple organs. Patients with triple-positive antiphospholipid syndrome have elevated lupus anticoagulant, B2GPI and anticardiolipin antibodies and higher risk of thrombosis. We present a case of fatal CAPS with thrombotic storm in a patient with triple-positive APS. CASE PRESENTATION: A 32 year-old female with history of immune thrombocytopenic purpura, MSSA endocarditis with mitral valve repair, APS on apixaban, cerebral cavernous malformation, and a chronic left ischemic middle cerebral artery stroke presented with hypotension, encephalopathy, lethargy, and cool and mottled lower extremities for 3 weeks. Labs revealed leukocytosis, kidney injury, lactic acidosis, and elevated BNP. She developed respiratory failure and was intubated. CT head was negative. CTA showed an occlusion of the distal right common iliac artery. Arterial ultrasound confirmed occlusion of right popliteal arteries and right calf veins. She was started on a heparin drip. Transesophageal echocardiography revealed RV failure and mitral stenosis with vegetations consistent with endocarditis. Due to cardiogenic shock from RV failure, venoarterial ECMO support was initiated. Given her triple positive APS, there was concern for CAPS. Steroids and plasmapheresis were started. Patient underwent fasciotomy and thrombectomy of the right lower extremity with improved blood flow, but without pulses post procedure. Despite treatment, patient's neurological status began to worsen with no pupil, oculocephalic or cough reflex. Repeat CT head showed new bilateral middle cerebral artery infarcts. Subject to discussions with family, a comfort care approach was pursued and the patient died. DISCUSSION: Diagnosis of this potentially fatal entity with a 30% mortality rate is based on clinical suspicion in patients with evidence of multiorgan failure, multiple thromboses, small vessel occlusion and laboratory confirmation of antiphospholipid antibodies. Early treatment with anticoagulation, steroids, and plasma exchange is critical and can lead to improved recovery with survival rate 71% compared to 25% in those who receive no therapy. If expertise and capabilities are not available, transfer to another facility is indicated. Addressing thrombosis risk factors is important in preventing further thrombosis and possibly progression to CAPS in APS patients. CONCLUSIONS: Our case underscores the importance of maintaining a high index of suspicion for early diagnosis and treatment of CAPS to prevent severe morbidity and mortality. REFERENCE #1: Or Carmi, Maya Berla, Yehuda Shoenfeld & Yair Levy (2017) Diagnosis and management of catastrophic antiphospholipid syndrome, Expert Review of Hematology, 10:4, 365-374 REFERENCE #2: Ricard Cervera, Ignasi Rodríguez-Pintó, Gerard Espinosa. The diagnosis and clinical management of the catastrophic antiphospholipid syndrome: A comprehensive review, Journal of Autoimmunity, Volume 92, 2018, Pages 1-11, ISSN 0896-8411 REFERENCE #3: Espinosa G, Bucciarelli S, Asherson RA, Cervera R. Morbidity and mortality in the catastrophic antiphospholipid syndrome: pathophysiology, causes of death, and prognostic factors. Semin Thromb Hemost. 2008 Apr;34(3):290-4. doi: 10.1055/s-0028-1082274. PMID: 18720310. DISCLOSURES: No relevant relationships by Weston Bowker No relevant relationships by Faraz Damghani No relevant relationships by Ryan Farhat No relevant relationships by Abdul Ahad Khan No relevant relationships by Sumit Patel No relevant relationships by Nehan Sher No relevant relationships by Suresh Uppalapu