PRESUMED CATASTROPHIC ANTIPHOSPHOLIPID SYNDROME

Abstract

SESSION TITLE: Fellows Critical Care Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: October 18-21, 2020 INTRODUCTION: Antiphospholipid antibodies (aPLs) are associated with recurrent early pregnancy loss and arterial as well as venous thrombosis. A small subset of patients develop a life-threatening form of the disease with widespread thrombosis and multiorgan failure, called catastrophic antiphospholipid syndrome (CAPS). CASE PRESENTATION: A 26-year-old female, 10 weeks pregnant, with a history of 2 prior miscarriages presented with hyperemesis gravidarum. She had a pulseless electrical activity cardiac arrest in the ER with return of spontaneous circulation after 15 minutes of advanced cardiovascular life support (ACLS). Left heart catheterization showed no evidence of coronary artery disease. Transthoracic echocardiogram showed preserved ejection fraction and no other abnormality. CT angiogram of the chest ruled out pulmonary emboli but showed diffuse bilateral airspace opacities, concerning for acute respiratory distress syndrome (ARDS). Patient developed acute kidney injury, acute right lower extremity arterial thrombosis and compartment syndrome requiring fasciotomy, upper extremities superficial venous thrombi, fetal loss and spontaneous abortion. Working diagnosis of CAPS was made; aPLs, anti-cardiolipin and anti-beta2 glycoprotien-1 antibodies levels ordered and patient started on IV heparin infusion, IV glucocorticoids and plasmapheresis followed by IV immunoglobulins (IVIG). Anticoagulation was held intermittently due to acutely worsening thrombocytopenia with low fibrinogen levels concerning for disseminated intravascular coagulation (DIC), and bleeding around the fasciotomy site, hematuria and vaginal bleeding (workup for heparin induced thrombocytopenia remained negative). Cryoprecipitate and blood transfusions were required. Acute respiratory failure resolved with low tidal volume ventilation, prone positioning and aggressive diuresis. Tissue biopsies for histopathology were deferred. Patient was discharged to inpatient rehab after prolonged hospital stay. DISCUSSION: Approximately 1% of antiphospholipid syndrome patients develop CAPS. Lungs are the second most common organs involved with ARDS being the most common complication. Mortality was 37% in a cohort of 500 patients. Management of CAPS includes aPL testing including lupus anticoagulant, antibeta-2 glycoprotein-1 and anticardiolipin antibodies without delaying empiric treatment. Combination therapy with glucocorticoid, heparin, and plasmapheresis or IVIG rather than single agents is recommended. CONCLUSIONS: Differential diagnosis of CAPS includes DIC which usually presents with small vessel thrombosis but has been reported to cause arterial thrombosis as well, and for which the patient was treated once the platelet counts dropped later. However, with the patient’s history and clinical presentation and given the high mortality rate, we decided to start aggressive empiric therapy after having sent aPL testing. Reference #1: Cervera R.: Antiphospholipid syndrome. Thromb. Res. 2017; 151: pp. S43-S47 Reference #2: Cervera R., Piette J.-C., Font J., Khamashta M.A., Shoenfeld Y., Camps M.T., et al: Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum. 2002; 46: pp. 1019-1027 Reference #3: Bucciarelli S., Espinosa G., Cervera R., Erkan D., Gómez-Puerta J.A., Ramos-Casals M., et al: Mortality in the catastrophic antiphospholipid syndrome: causes of death and prognostic factors in a series of 250 patients. Arthritis Rheum. 2006; 54: pp. 2568-2576 DISCLOSURES: No relevant relationships by Priyanka Bhat, source=Web Response no disclosure on file for Shafaq Ejaz; No relevant relationships by Ahmed Qadir, source=Web Response No relevant relationships by karan Singh, source=Web Response No relevant relationships by Rodney Steff, source=Web Response