doi: 10.2169/internalmedicine.4958-20
Intern Med 59: 3225-3227, 2020
http://internmed.jp

【 CASE REPORT 】

Isolated Cerebral Vasculitis in the Unilateral Middle
Cerebral Artery in a Case with SLE
Sho Takeshita, Toshiyasu Ogata, Jun Tsugawa and Yoshio Tsuboi

Abstract:
A 47-year-old woman, who was diagnosed to have systemic lupus erythematosus (SLE), was admitted because she suffered a severe ischemic stroke three weeks after experiencing a transient attack of aphasia.
Diffusion-weighted MRI revealed high intensity at the borderzone of the middle cerebral artery (MCA), while
the proximal portion of the left MCA was occluded with its vascular wall enhanced by gadolinium. Intravenous methylprednisolone and heparin were administrated without any symptomatic benefit. She developed severe right hemiparesis with aphasia. Isolated cerebral vasculitis in the large vessel has been rarely reported in
SLE patients. The presence of an enhanced vascular wall in the MRI with gadolinium could support the diagnosis.
Key words: systemic lupus erythematosus, SLE, vasculitis, cerebral large artery, acute cerebral infarction
(Intern Med 59: 3225-3227, 2020)
(DOI: 10.2169/internalmedicine.4958-20)

Introduction
Systemic lupus erythematosus (SLE) is an autoimmune
disease involving multiple organs, including the central
nervous system (CNS). Neurological complications in patients with SLE are known as neuropsychiatric SLE
(NPSLE). Brain ischemia due to CNS vasculitis has also occurred in such patients (1). Although vasculitis of the cerebral small-vessels is well known, such an occurrence in
large-vessels has rarely been reported in SLE patients (2-5).
We herein report a patient with SLE presenting with a progressive ischemic stroke due to vasculitis isolated in the unilateral middle cerebral artery (MCA).

Case Report
A 47-year-old woman was admitted to our emergency
room because of sudden development of aphasia. She presented with photosensitivity, arthritis, anemia and proteinuria
at 27 years of age and was diagnosed to have lupus nephritis [World Health Organization (WHO) type III] based on a
renal biopsy, leading to the administration of intravenous
methylprednisolone and subsequent oral glucocorticoid and

cyclosporine. Recently, she was administered 5 mg per day
of oral prednisolone and here symptoms were well controlled. She had no family history of neurological disorders
and no history of smoking nor any medical history of hypertension, diabetes mellitus, or dyslipidemia. A neurological
examination on admission revealed non-fluent aphasia and
mild weakness in the right arm, which was estimated as a
score of 2 on the National Institute of Health Stroke Scale
(NIHSS). MRI with diffusion-weighted imaging (DWI)
identified high signal intensity at the border around the left
MCA (Fig. 1). Magnetic resonance angiography (MRA) revealed stenosis in her left MCA (Fig. 1). A brain infarction
was confirmed and oral cilostazol was initiated. The vascular
wall of the left MCA was clearly enhanced on gadoliniumenhanced MRI, thus suggesting vascular wall inflammation,
which thus led to a high suspicion of vasculitis. SLE-related
inflammatory reactions such as fever, discoid rash, or cheek
and lymph-node swelling were absent. Laboratory studies
showed elevated white blood cell of 14.2×103/μL, C-reactive
protein level of 4.09 mg/dL and erythrocyte sedimentation
rate of 58 mm/hour with decrease level of hemoglobin of
9.6 g/dL. Platelet count of 316×103/μL were within the normal limits. An immunological test showed a positive finding
for antinuclear antibodies of 26.6 by ELISA and double-

Department of Neurology, Faculty of Medicine, Fukuoka University, Japan
Received: March 30, 2020; Accepted: June 4, 2020; Advance Publication by J-STAGE: July 21, 2020
Correspondence to Dr. Yoshio Tsuboi, tsuboi@cis.fukuoka-u.ac.jp

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Day 2

DOI: 10.2169/internalmedicine.4958-20

Day 10

Day 36

Day 37

Day 58

MRA

DWI
Axial

Figure 1. Time course of the MRI findings 2, 10, 36, 37, and 58 days after the first admission. Upper: Time-of-flight magnetic resonance angiography (TOF-MRA) of the cerebral arteries. Severe
narrowing was observed in the proximal portion of the left middle cerebral artery (MCA) on day 2,
which was improved on day 10 (arrow). On day 36 and thereafter, severe narrowing recurred with
the distal arteries observed to be attenuated. Lower: Axial findings via diffusion weighted imaging
(DWI). A small high intensity area (HIA) was found in her left frontal lobe on day 2 (arrow). On day
36, other HIAs were detected in her frontal lobe (arrow) in addition to her corona radiate and parietal
lobe, indicating the recurrence of a brain infarction.

Day 5

Day 38

Day 52

Figure 2. The time course of contrast-enhanced T1-CUBE findings 5, 38, 52 after the first admission. Day 5: The proximal portion of her left MCA exhibited vascular wall thickness with enhancement (arrow). Day 38: The vascular wall thickness and enhancement extended from the cervical
portion of her internal carotid artery (ICA) to the horizontal portion of MCA (arrows). Day 52: Her
left ICA had strong enhancement with obvious wall thickening (arrow). MCA: middle cerebral artery

stranded DNA antibodies of 23.8 IU/mL. Anti SS-A/SS-B
antibodies, anti-neutrophil cytoplasmic antibody, anticardiolipin antibody test and a lupus anticoagulant test were
negative. The levels of serum complements including CH50
(58 U/mL; normal 32-58 U/mL), C3 (110 mg/dL; normal
73-138 mg/dL), and C4 (26 mg/dL; normal 11-31 mg/dL)
were normal. The stenosis of the left MCA exhibited an improvement on day 10 and her neurological deficit disappeared. She was discharged from the hospital on day 17.
Three weeks later, she was again admitted due to transient
motor aphasia and right facial palsy. DWI indicated an addi-

tional acute ischemic stroke around her left MCA (Fig. 1).
MRA indicated that the left MCA was nearly completely
occluded and gadolinium-enhanced MRI revealed thickening
of the vascular wall from the cervical portion of the internal
carotid artery (ICA) to the horizontal portion of the MCA
(Fig. 2), indicating a progression of vasculitis leading to the
left MCA occlusion. An examination revealed normal cerebrospinal fluid (CSF) without any oligoclonal bands, however, the interleukin-6 (IL-6) level of the cerebrospinal fluid
was elevated (24.9 pg/mL; normal 0-4 pg/mL). The intravenous administration of methylprednisolone (1,000 mg/day, 3

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DOI: 10.2169/internalmedicine.4958-20

days) with heparinization was initiated. However, her nonfluent aphasia and right hemiparesis worsened after two
days, and her NIHSS score rose to 10. A re-examination via
DWI revealed significant enlargement of the ischemic lesion
in her left corona radiate. Therefore, treatment with a high
dose of oral steroids and cyclophosphamide was initiated,
and a follow-up study of CSF indicated the IL-6 level to
have decreased to 2.9 pg/mL. However, her neurological
deficits did not improve. She had severe motor aphasia and
right hemiparesis and an NIHSS score of 15 on day 41. The
imaging findings were unchanged, and she was transferred
to another hospital for rehabilitation on day 62.

Discussion
According to the American College of Rheumatology
classification criteria, our case is classified as having cerebrovascular disease. It is reported that, among NPSLE patients, 30-40% are complicated with ischemic stroke. In addition, the three subtypes of vasculopathy are classified by
neuroimaging analyses, including inflammatory-like lesions,
small-vessel disease (SVD), and large-vessel disease (LVD).
Among these, the most common type is SVD, which accounts for 60% of cases with NPSLE (2). SVD is an intrinsic disorder of the brain-perforating arteries, characterized
by white matter and subcortical legions of the brain. In contrast, LVD in cases with NPSLE is relatively rare. According
to a previous article, 13% of cases with NPSLE have been
classified to the LVD type (2). The cause of LVD in SLE
cases may vary, including circulating lupus-anticoagulant,
Libman-Sacks endocarditis, sepsis, atherosclerotic disease or
vasculitis. Previously, MRA or cerebral angiography was
performed for the evaluation of LVD vasculitis (3-5). On the
other hand, contrast-enhanced MRI with the T1-CUBE sequence, which we used in this case, can clearly exhibit the
thickening of the vessel wall with enhancement. These findings may provide evidence of inflammation in the vessel
wall, thus indicating a diagnosis of cerebral vasculitis (6). In
such cases of cerebral vasculitis, we believe the possible etiologies to include infection, giant-cell arteritis, granulomatosis with polyangiitis, Behçet disease, Sjögren syndrome and
drug-induced arteritis. However, all of these etiologies were
ruled out based on her clinical features and laboratory findings. Therefore, we concluded that the patient suffered from
large vessel vasculitis in the central nervous system related
to SLE when making the diagnosis (7).
Patients with LVD vasculitis related to SLE should undergo strong immunotherapy as early as possible, however,
no useful biomarkers have been established for the early diagnosis or treatment efficacy. Although the effectiveness of
rituximab therapy has been reported for the refractory neuropsychiatric lupus (8), there is little evidence that it would
have any beneficial effect on the LVD vasculitis caused by

SLE. An increased level of IL-6 in cerebrospinal fluid could
be a biomarker for the activity of NPSLE. It has been recognized that the IL-6 level has a strong association with a
diagnosis of NPSLE, the activity of SLE vasculitis, clinical
deterioration, and treatment response (9). In our case, an increased IL-6 level was obvious at the second hospitalization,
thus indicating the activity of SLE vasculitis. Stroke could
also have caused an elevation of IL-6 in our case (10). However, because there is no evidence of a disruption of the
blood-brain barrier, such as increase of protein or albumin in
the CSF, the elevation of IL-6 in our case most likely reflects the activity of SLE vasculitis. Our case suggests that
increased CSF IL6 levels associated with vascular wall
thickening and enhancement on contrast-enhanced MRI may
suggest a possible diagnosis of SLE-associated LVD.
Conclusion

We experienced a case of ischemic stroke with an occluded MCA due to SLE vasculitis. This case was an atypical NPSLE in that the patient demonstrated vasculitis of a
large cerebral artery. The IL-6 levels in the CSF and
gadolinium-enhanced MRI might play an important role in
predicting the clinical course and treatment response.
The authors state that they have no Conflict of Interest (COI).

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