Clinical Communications: Adults
Seronegative Catastrophic Antiphospholipid Syndrome in a Young Female Presenting with a Headache

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Abstract

Background

Catastrophic antiphospholipid syndrome (CAPS) is a rare disease that causes rapid vascular occlusion in multiple organ systems. Initial presentation varies depending on the organs affected. Although headache is a common complaint in the emergency department (ED), it is a very rare presentation of CAPS.

Case Report

A 43-year-old previously healthy woman presented to the ED with severe headache. Subarachnoid hemorrhage was excluded and she was discharged home. She returned 36 h later with diabetic ketoacidosis, hyperthyroidism, and thrombosis in her cerebral venous sinus, aorta and splenic artery. She was treated with heparin, steroids, plasmapharesis, and i.v. immunoglobulin, after which she improved. This constellation of symptoms is highly suggestive of CAPS initiated by a polyglandular autoimmune syndrome, despite negative serology.

Why Should an Emergency Physician Be Aware of This?

Although a rare cause of headache, CAPS is a potentially fatal disease that requires early identification and initiation of appropriate treatment.

Introduction

One unusual cause of headache with significant clinical consequences is catastrophic antiphospholipid syndrome (CAPS). Antiphospholipid antibody syndrome (APS) is an autoimmune disease characterized by antibodies against the antiphospholipid complex, which presents as thrombosis. CAPS is a very rare sequela, occurring in < 1% of patients with APS, in which vascular occlusion occurs in three or more locations simultaneously (Table 1) 1, 2, 3. CAPS has a high morbidity and mortality, with death occurring in up to 44% of patients (4). Initial presentations vary but rarely occur as cerebral venous thrombosis, as in our case. Clinical identification and early treatment by emergency medicine physicians remains essential.

Section snippets

Case Report

A 43-year-old woman from Afghanistan with recent onset of vitiligo but no other known medical history first presented to the emergency department (ED) with the “worst headache of her life.” A noncontrast computed tomography (CT) scan of her head and lumbar puncture (LP) were performed to rule out subarachnoid hemorrhage. Both were unremarkable. The complete blood count and basic metabolic panel were within normal limits except for a serum glucose of 337 mg/dL. Her headache improved with i.v.

Discussion

APS is defined by one or more episodes of arterial, venous, or small vessel vascular thrombosis or pregnancy morbidity with a positive antiphospholipid antibody (5). To confirm the diagnosis, the lupus anticoagulant, anticardiolipin antibody, or anti-β2 glycoprotein-I antibody should be positive on two or more occasions 12 weeks apart. The catastrophic variant, CAPS, was first described by Asherson in 1992 and occurs in < 1% of patients with APS (6). Characteristic features of CAPS include

Why Should an Emergency Physician Be Aware of This?

A healthy female entered into the ED with the “worst headache of her life” and within the span of 3 days became critically ill with DKA, hyperthyroidism, and venous/arterial thromboembolism affecting her brain, aorta, and spleen. Prompt medical diagnosis with administration of steroids, anticoagulation, plasmapheresis, and IVIG stabilized the patient's thrombotic state (20). Most important to the emergency medicine provider is that the decision to treat was made solely on clinical evidence.

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