WSO - April 2023 - 383

1150349
WSO
International Journal of StrokeMittal et al.
Review
Antiphospholipid syndrome,
antiphospholipid antibodies, and stroke
Prabal Mittal1,2, Graziella Quattrocchi3,4, Ibrahim Tohidi-Esfahani1,2,
Zara Sayar1,5, Arvind Chandratheva3,6 and Hannah Cohen1,2
International Journal of Stroke
2023, Vol. 18(4) 383 -391
© 2023 World Stroke Organization
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DOI: 10.1177/17474930221150349
journals.sagepub.com/home/wso
https://doi.org/10.1177/17474930221150349
Abstract
Antiphospholipid syndrome (APS) is a prothrombotic autoimmune disease with heterogeneous clinicopathological
manifestations and is a well-established cause of acute ischemic stroke (AIS) and transient ischemic attack (TIA), particularly
in younger patients. There is growing recognition of a wider spectrum of APS-associated cerebrovascular lesions,
including white matter hyperintensities, cortical atrophy, and infarcts, which may have clinically important neurocognitive
sequalae. Diagnosis of APS-associated AIS/TIA requires expert review of clinical and laboratory information. Management
poses challenges, given the potential for substantial morbidity and recurrent thrombosis, additional risk conferred
by conventional cardiovascular risk factors, and limited evidence base regarding optimal antithrombotic therapy for
secondary prevention. In this review, we summarize key features of APS-associated cerebrovascular disorders, with
focus on clinical and laboratory aspects of diagnostic evaluation. The current status of prognostic markers is considered.
We review the evidence base for antithrombotic treatment in APS-associated stroke and discuss uncertainties, including
the optimal intensity of anticoagulation and efficacy of direct oral anticoagulants. Clinical practice recommendations are
provided, covering antithrombotic treatment, supportive management, and options for anticoagulant-refractory cases,
and we highlight the benefits of adopting a considered, multidisciplinary team approach.
Keywords
Antiphospholipid syndrome, antiphospholipid antibodies, ischemic stroke, cerebrovascular disorders, cognitive
impairment, antithrombotic
Received: 7 July 2022; accepted: 9 November 2022
Search strategy and selection criteria
The following search strategy was used in PubMed, limiting to
English language articles from January 1990 to March 2022.
- Search terms: [ " antiphospholipid syndrome " OR
" antiphospholipid antibodies " ] AND [ " stroke " OR
" cerebrovascular " ]
This was supplemented with recommendations from senior
authors and citation searching from key articles. The final
reference list was selected according to relevance to the
broad scope of this review.
Introduction
Antiphospholipid syndrome (APS) is an acquired autoimmune
thrombophilia with an estimated incidence and prevalence
between 1 and 2 cases per 100,000 and 40 and 50
cases per 100,000, respectively.1 Cardinal features
are arterial, venous, or microvascular thrombosis and/or
obstetric morbidity, including recurrent early miscarriage,
late pregnancy loss, stillbirth, and placental insufficiency.
The diagnosis requires coexistence with persistent
1 Department of Haematology, University College London Hospitals
NHS Foundation Trust, London, UK
2 Haemostasis Research Unit, Department of Haematology, University
College London, London, UK
3 Comprehensive Stroke Service, University College London Hospitals
NHS Foundation Trust, London, UK
4 Department of Neurology, North Middlesex NHS Trust, London, UK
5 Department of Haematology, Whittington Health NHS Trust,
London, UK
6 UCL Stroke Research Centre, Department of Brain Repair and
Rehabilitation, UCL Institute of Neurology and the National Hospital
for Neurology and Neurosurgery, London, UK
Corresponding author:
Prabal Mittal, Department of Haematology, University College London,
72 Huntley St, London, WC1E 6DD, UK.
Email: prabal.mittal@nhs.net
International Journal of Stroke, 18(4)
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