(421) A Newly Diagnosed Case of Catastrophic Antiphospholipid Antibody Syndrome in a 60-Year-Old

Introduction: Asherson’s syndrome, a rare and fatal catastrophic antiphospholipid syndrome variant, causes accelerated multifocal widespread intravascular thrombosis and multiorgan ischemia. It affects 1 % of APS patients, often young females and is characterized by ‘triple aPL positivity,’ and high anticardiolipin and anti-beta2 glycoprotein antibodies.

Description: We present a case of a 60-year-old man presented with severe shortness of breath, orthopnea, and bilateral edema. He had atrial flutter with a rapid heart rate, elevated NT proBNP (48,000 pg/ml), troponin (45 ng/l), and abnormal liver function tests. Abdominal ultrasound revealed an IVC thrombus. CTPE showed several segmental and subsegmental bilateral pulmonary emboli with multifocal infarcts. He was started on heparin, diuretics, and digoxin. Echocardiogram showed reduced EF of 5 to 10%, left and right ventricular apical thrombus, and diastolic dysfunction. Two days later, he developed aphasia and right-sided facial droop, with MRI indicating left frontal lobe stroke. The patient suffered two cardiac arrests; the first showed wide-complex tachycardia, and the second was ventricular fibrillation within next two days. Cardiac catheterization demonstrated 60-70% lesion in the mid-distal LAD. He had another cardiac arrest but was stabilized with AICD. Hypercoagulable work-up suggested antiphospholipid syndrome. He was treated with Solu-Medrol, IVIG, and transferred for plasmapheresis. Follow-up within a year showed EF improved to 55-60%.

Discussion: Around half of APS patients may first present with catastrophic events, often due to non-compliance with anticoagulation. Predisposing factors include infection, surgery, or SLE. Rapid clinical deterioration followed by multiple system involvement with microangiopathic hemolytic anemia and thrombocytopenia form significant clinical manifestations of the syndrome. Early recognition and aggressive treatment with glucocorticoids, plasma exchange, IVIG, and anticoagulation are crucial, as mortality can reach 30%. Awareness of pathophysiology and recognition of this thrombotic storm is of utmost importance to ensure timely management especially in older patients. This is crucial due to lower risk of autoimmune conditions in older age which may remain unrecognised.