Neurologic Manifestations of Catastrophic Antiphospholipid Syndrome

Understanding of antiphospholipid antibody syndrome (APS), associated neurological manifestations, and disease-directed treatment has grown considerably over the last decade. Herein, we critically review the current and high-yield literature related to the pathophysiology, neurological presentations, and management of APS with particular emphasis on the rare and more fatal subset of APS, catastrophic antiphospholipid syndrome (CAPS).

APS may manifest with a variety of neurologic syndromes, with cerebrovascular disease representing the most commonly encountered presentation. Diagnostic evaluation and treatment are often tailored to the specific presentation, with suspicion and testing for antiphospholipid antibodies recommended when neurologic presentations occur atypically or in younger individuals. In CAPS, which is more rapidly progressive with multiorgan involvement, potential alternative microangiopathic syndromes should be carefully considered in the differential diagnosis. To date, anticoagulation with vitamin K antagonists remains the mainstay of therapy in APS while triple therapy with anticoagulation, corticosteroids, and plasma exchange is standard of care in CAPS. Immunotherapy has shown early promise in refractory cases. APS is an autoimmune clinical syndrome with neurologic presentations classically characterized by vascular thrombosis, though recent understandings suggest additional direct immune-mediated phenomena. Our understanding of the underlying pathogenic mechanisms of APS continues to grow and will continue to influence our therapeutic approaches.