Stroke in lupus and APS
LCD de Amorim et al.
6
months after the event. Minor strokes are strokes
with clinical presentation of mild symptoms, pre-
senting with a low score on the National Institutes
of Health Stroke Scale (NIHSS) and distributed in
separate subitems.24 SLE patients with a prior TIA
history are at risk of ischaemic stroke of 57%.25
The true prevalence of TIA is underestimated,
because a large proportion of patients fail to
report it to a healthcare provider, as is also the
case with minor stroke.4 Valve disease, especially
in the left chambers, increases the risk when asso-
ciated with disease activity. Moreover, the presence
of aPL may be seen in 6.3% of cases of TIA in
SLE.1,4 The most common clinical manifestations
of TIA include muscle weakness, language deficits,
amaurosis fugax, vertigo, and transient global
ischaemia (Table 1).
Ischaemic stroke
Ischaemic stroke represents an irreversible or par-
tially reversible neurologic deficit due to failure or
acute vascular occlusion, corresponding to 85% of
the cases of stroke,6 and the prevalence of stroke in
SLE varies from 2% to 19%.11,23,26 The risk of
ischaemic stroke is twice as frequent in individuals
with SLE and involves more than the traditional
Framingham risk factors. Peculiarities such as the
presence of vasculitis, associated APS and/or posi-
tive aPL autoantibodies (present in 9.4% of cases),
endocarditis, Libman-Sacks endocarditis, hyper-
viscosity (increased homocysteine levels), genetic
polymorphism (presence of allele GT20), and
hypertension determine the increased risk of an
ischaemic cerebrovascular event.4,7 Reinforcing
this finding, a study27 found the risk of stroke
and acute myocardial infarction (AMI) to be 1.5
times higher in males with b2GPI-dependent
Table 1 Prevalence and clinical manifestations of cerebrovascular disease in SLE and APS
Clinical manifestations Prevalence
Transient ischaemic attack Underestimated in SLE
APS 11%
Stroke SLE 2%–19%
APS 19.8%
Ischaemic stroke 19% in SLE and APS
Haemorrhagic stroke Very low (0.4%)
Subarachnoid haemorrhages 5% of all strokes
Central venous thrombosis – It usually presents as a refractory headache, sometimes associated with
APS: antiphospholipid syndrome; SLE: systemic lupus erythematosus; TIA: transient ischemic attack.
531
immunoglobulin (Ig)G anticardiolipin antibodies
than in those without, and another study28 demon-
strated that high serum concentrations of anticar-
diolipin antibodies, regardless of other
cardiovascular risk factors, were an important pre-
dictor of the risk of future stroke and TIA in
females but not in males.
Ischaemic stroke often affects patients less than
50 years of age, and in the first five years of disease,
particularly during the first year. This is due to sys-
temic inflammatory processes leading to early ath-
erosclerosis.1,4,7,8 The risk of ischaemic stroke is
twice the first year after hospitalization that of
non-carriers of autoimmune diseases, and remains
high for 10 years; the recurrence rate of ischaemic
stroke is 35% of cases.26
According to Mikdashi et al.,23 SLE patients
who develop ischaemic stroke often have an
NIHSS score  6 with mortality rates ranging
from 15% to 25%. In APS, stroke is the most
common and severe complication.29 Patients with
stroke and aPL positivity are younger and are more
likely to be female than are aPL-negative stroke
patients.17,18 In the Euro-Phospholipid Project
Group study that investigated 1000 APS patients,
19.8% presented with stroke, 11.1% presented with
TIA, and 5.5% presented with AMI.30
Some authors have suggested that Sneddon’s
syndrome should be included in the neurological
manifestations of APS and SLE, mainly because
more than 40% of the patients with Sneddon’s syn-
drome have aPL,31 and this syndrome is character-
ized by the development of an ischaemic
cerebrovascular disease associated with livedo reti-
cularis. Patients with APS can present main cere-
bral artery territory infarction, such as middle
cerebral artery territory infarction, whereas
Key messages
SLE patients with prior TIA history at risk of ischaemic stroke up to
57%.
Stroke affects often patients less than 50 years, in the first five years of
disease, particularly during the first year.
Vasculitis, APS secondary and/or positive autoantibody (present in
9.4% of cases) and hypertension determine the increased risk of
ischaemic cerebrovascular event.
It occurs more frequently in patients over 50 years old with hyperten-
sion and more common in SLE patients than non-SLE patients.
Aneurysm rupture is the most common cause.
an epileptic seizure, focal deficits or intracranial hypertension
syndrome.
Lupus