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Purpose of Review <p>Antiphospholipid antibody syndrome (APS) is an autoimmune disorder marked by sustained presence of antiphospholipid antibodies (aPL) and an increased risk of arterial and venous thrombotic events, particularly cerebrovascular events. This review summarizes the clinical features, pathophysiology, and diagnostic approach to cerebrovascular disease in APS while also examining current preventative and acute treatment trends. We include literature on primary and secondary prevention strategies as well as considerations of acute treatments including IV thrombolysis and mechanical thrombectomy.</p> Recent Findings <p>Diagnostic evaluation and treatment of APS are often tailored to specific presentations, with suspicion and testing for aPLs recommended when neurologic presentations occur atypically or in younger individuals. In catastrophic antiphospholipid syndrome (CAPS), which is more rapidly progressive with multiorgan involvement, potential alternative microangiopathic syndromes should be carefully considered in the differential diagnosis. To date, antiplatelet monotherapy remains the mainstay of primary prevention in APS, while anticoagulation with vitamin K antagonists is standard of care for secondary stroke prevention. In CAPS, triple therapy with anticoagulation, corticosteroids, and plasma exchange has been shown to significantly reduce mortality. Immunotherapy has shown early promise in refractory cases.</p> Summary <p>APS may manifest in a variety of neurologic syndromes, though cerebrovascular disease represents the most commonly encountered phenomenon. With ongoing research, our understanding of the underlying pathogenic mechanisms of this disease continues to grow and will continue to influence our therapeutic approaches. For now, anticoagulation with vitamin K antagonists remains the standard of care in APS-associated neurologic disease. Further studies will help clarify the pathogenic underpinnings of APS and enhance primary and secondary prevention strategies, with the ultimate aim to reduce morbidity and mortality within this population.</p>

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Update on Stroke Prevention and Treatment in Patients with Antiphospholipid Antibody Syndrome

  • 期刊论文

  • 发表日期：

    2025年1月29日

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  • Helena K. Xeros,
  • Molly C. Bates,
  • Rafid Mustafa

摘要

Purpose of Review

Antiphospholipid antibody syndrome (APS) is an autoimmune disorder marked by sustained presence of antiphospholipid antibodies (aPL) and an increased risk of arterial and venous thrombotic events, particularly cerebrovascular events. This review summarizes the clinical features, pathophysiology, and diagnostic approach to cerebrovascular disease in APS while also examining current preventative and acute treatment trends. We include literature on primary and secondary prevention strategies as well as considerations of acute treatments including IV thrombolysis and mechanical thrombectomy.

Recent Findings

Diagnostic evaluation and treatment of APS are often tailored to specific presentations, with suspicion and testing for aPLs recommended when neurologic presentations occur atypically or in younger individuals. In catastrophic antiphospholipid syndrome (CAPS), which is more rapidly progressive with multiorgan involvement, potential alternative microangiopathic syndromes should be carefully considered in the differential diagnosis. To date, antiplatelet monotherapy remains the mainstay of primary prevention in APS, while anticoagulation with vitamin K antagonists is standard of care for secondary stroke prevention. In CAPS, triple therapy with anticoagulation, corticosteroids, and plasma exchange has been shown to significantly reduce mortality. Immunotherapy has shown early promise in refractory cases.

Summary

APS may manifest in a variety of neurologic syndromes, though cerebrovascular disease represents the most commonly encountered phenomenon. With ongoing research, our understanding of the underlying pathogenic mechanisms of this disease continues to grow and will continue to influence our therapeutic approaches. For now, anticoagulation with vitamin K antagonists remains the standard of care in APS-associated neurologic disease. Further studies will help clarify the pathogenic underpinnings of APS and enhance primary and secondary prevention strategies, with the ultimate aim to reduce morbidity and mortality within this population.