Lupus (2017) 26, 529–536

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SPECIAL ARTICLE

Stroke in systemic lupus erythematosus and antiphospholipid

syndrome: risk factors, clinical manifestations, neuroimaging, and
treatment
LCD de Amorim1, FM Maia1 and CEM Rodrigues1,2
1
University of Fortaleza (Unifor), Fortaleza, Brazil; and 2Federal University of Ceará, Fortaleza, Brazil

Neurologic disorders are among the most common and important clinical manifestations
associated with systemic lupus erythematosus (SLE) and antiphospholipid syndrome (APS),
mainly those that affect the central nervous system (CNS). Risk of cerebrovascular events in
both conditions is increased, and stroke represents one of the most severe complications, with
an incidence rate between 3% and 20%, especially in the first five years of diagnosis. This
article updates the data regarding the risk factors, clinical manifestations, neuroimaging, and
treatment of stroke in SLE and APS. Lupus (2017) 26, 529–536.

Key words: Stroke; systemic lupus erythematosus; antiphospholipid syndrome; Hughes’

syndrome; antiphospholipid antibodies; neurological manifestations

Introduction aPL, which include anticardiolipin, lupus anti-

coagulant, and anti-beta 2-glycoprotein I
Systemic lupus erythematosus (SLE) is a chronic (b2GPI).4 Approximately 36% of APS patients
systemic inflammatory disease, of autoimmune also have SLE, and the combination of these con-
origin, that may affect the central nervous system ditions can amplify the risk of aPL-mediated
(CNS), and this association is responsible for CVEs.4
increased morbidity and mortality.1,2 The risk of With the increase in life expectancy over the past
cerebrovascular events (CVEs) is increased in 50 years, SLE patients have a survival rate of
SLE, and stroke represents one of the most severe approximately 92% in 10 years. It is estimated
complications, with an incidence rate between 3% that for every aged year, the cardiovascular risk
and 20%, especially in the first five years of dis- increases by 3% and that CEVs may be responsible
ease.3 Stroke can be caused by ischaemic or haem- for 20% to 30% of the mortality in these
orrhagic events, and its occurrence can be explained patients.5,6 Despite important advances in the
by many mechanisms such as disease-specific understanding of SLE and APS, neurological mani-
hypercoagulable state (presence of antiphospholi- festations continue to pose diagnostic and thera-
pid antibodies (aPL)), hyperhomocysteinemia, peutic challenges for practicing physicians.7
lupus disease activity, cerebral vasculitis, emboli A meta-analysis has indicated that individuals
from Libman-Sacks endocarditis, accelerated ath- with SLE have a two-fold higher risk of ischaemic
erosclerosis, and traditional stroke risk factors stroke, a three-fold higher risk of intracerebral
(e.g. systemic arterial hypertension, hyperlipid- haemorrhage, and an almost four-fold higher risk
aemia, diabetes mellitus, and cigarette smoking).1,2 of subarachnoid haemorrhage (SAH) compared to
Antiphospholipid syndrome (APS) is an auto- the general population, and this risk is highest
immune disease characterized by recurrent throm- among individuals younger than 50 years of age.1
botic events, miscarriages, and thrombocytopenia, In APS, it has been suggested that more than 20%
and it is associated with the persistent presence of of strokes in patients younger than 45 years are
associated with APS.8
Because of the need for better understanding
Correspondence to: Carlos Ewerton Maia Rodrigues, Reumatologia,
Universidade de Fortaleza, Rua Dr Gilberto Studart, 955, Coco,
of the risk of stroke in SLE and APS and its
Fortaleza-Ceará 60192-095, Brazil. peculiarities in comparison to healthy individ-
Email: carlosewerton@hotmail.com uals, this review updates the data regarding the
! The Author(s), 2017. Reprints and permissions: http://www.sagepub.co.uk/journalsPermissions.nav 10.1177/0961203316688784