Neurological Manifestation of Anti Phospholipid Syndrome

1. Neurological Manifestation of
Antiphospholipid Syndrome
Ade Wijaya, MD – March 2019

2. Introduction
• An autoimmune antibody-mediated disorder defined by the occurrence of
thrombosis and/or pregnancy morbidity in presence of persistent
antiphospholipid antibodies (aPL)
• Incidence: 5 cases every 100,000 subjects/year
• Prevalence of 40–50 every 100,000 subjects
• Primary vs secondary (related to other autoimmune diseases) APS
Miyakis S, Lockshin MD, Atsumi T, Branch DW, Brey RL, Cervera R, et al. . International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. (2006) 4:295–306.
Schreiber K, Sciascia S, de Groot PG, Devreese K, Jacobsen S, Ruiz-Irastorza G, et al. Antiphospholipid syndrome. Nat Rev Dis Primers (2018) 4:18005
Garcia D, Erkan D. Diagnosis and management of the antiphospholipid syndrome. N Eng J Med. (2018)378:2010–21. 10.1056/NEJMra1705454

3. APS Diagnostic Criteria
Miyakis S, Lockshin MD, Atsumi T, Branch DW, Brey RL, Cervera R, et al. . International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. (2006) 4:295–306.

4. APS Pathogenesis
Fleetwood T, Cantello R, Comi C. Antiphospholipid syndrome and the neurologist: from pathogenesis to therapy. Frontiers in neurology. 2018;9.

7. Neurological Manifestation
Sanna G, D'Cruz D, Cuadrado MJ. Cerebral manifestations in the antiphospholipid (Hughes) syndrome.Rheumat Dis Clin North Am. (2006) 32:465–90.

8. Treatment
• Antiplatelet
• Anticoagulation
• Catastrophic APS: anticoagulants plus glucocorticoids plus plasma exchange (PE)
and/or intravenous immunoglobulins (IVIG)
• Rituximab
• Hydroxychloroquine
• Statins
• Immunosuppresant agents
Fleetwood T, Cantello R, Comi C. Antiphospholipid syndrome and the neurologist: from pathogenesis to therapy. Frontiers in neurology. 2018;9.
Ricarte IF, Dutra LA, Abrantes FF, Toso FF, Barsottini OG, Silva GS, de Souza AW, Andrade D. Neurologic manifestations of antiphospholipid syndrome. Lupus. 2018 Aug;27(9):1404-14.

10. Summary
• Rare and heterogeneous condition
• APS should always be considered in young patients with CVA, especially in
the absence of other vascular risk factors
• Thrombotic and immune-mediated
• Treatment: Anticoagulation with warfarin

11. THANK YOU