Rationale and Objective
Neuropsychiatric systemic lupus erythematosus (NPSLE) is a diagnostically challenging, severe, and life-threatening condition, which is currently lacking a “gold standard.” Our aim with this study is to look for magnetic resonance (MR) perfusion differences in NPSLE, SLE, and healthy control (HC) patients and correlate our findings with clinical parameters.
Materials and Methods
Twenty-four NPSLE patients, 21 SLE patients, and 21 HC underwent dynamic susceptibility contrast enhanced MR perfusion using a 3-T scanner. Nine prospectively selected intracranial regions of interest were placed in white and gray matter and the cerebral blood flow (CBF), cerebral blood volume (CBV), and mean transit time (MTT) values were calculated. Subjects underwent clinical evaluation with SLEDAI and serum antibodies.
Results
The SLE patients had higher CBF and CBV compared to the HC overall ( P = .01) and in specific areas ( P = .03–.048). SLE patients with signs of active disease (elevated SLEDAI and anti–double-stranded DNA) had significantly elevated CBV, CBF, and MTT in the posterior cingulate gyrus ( P = .01–.02). No significant difference was seen in the magnetic resonance perfusion measurements of NPSLE patients compared to SLE and HC, although the NPSLE patients also showed higher CBV variability compared to the SLE ( P = .0004) and HC cohort ( P < .0001).
Conclusion
SLE patients have increased CBV and CBF compared to healthy controls. The SLE patients with clinical markers for active disease have elevated CBV, CBF, and MTT in the posterior cingulate gyrus. NPSLE patients show increased variability in perfusion measurements, which may explain why susceptibility contrast enhanced MRI has not yet provided a specific target for NPSLE.
Introduction
Neuropsychiatric systemic lupus erythematosus (NPSLE) is a severe and life-threatening condition, reported to occur in 25%–70% of systemic lupus erythematosus (SLE) patients . NPSLE patients present with protean clinical manifestations such as: aseptic meningitis, stroke, transient ischemic attack, cerebral venous sinus thrombosis, delirium/dementia, demyelinating syndrome, headaches, movement disorders, psychosis, or mood/anxiety disorders .
NPSLE patients have a decreased quality of life and a worse prognosis compared to SLE patients . Therefore, it would be clinically and prognostically helpful to develop a “gold standard” to diagnose patients with NPSLE. Although several studies have described abnormal imaging findings and cortical atrophy in NPSLE patients, few studies have looked at dynamic perfusion in lupus patients. In contrast to the previously published studies, we wanted to see if lupus patients had focal areas of perfusion derangement and decided to use frontal white matter as a denominator/constant for our perfusion calculations .
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Materials and methods
Subjects
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Table 1
Demographic and Clinical Data
HC ( n = 19) SLE ( n = 18) NPSLE ( n = 21)P value Range
Mean ± SD Range
Mean ± SD Range
Mean ± SD Age 21.1–65.8
44.9 ± 14.3 23.5–64.7
43.1 ± 10.7 28.4–69.9
43.7 ± 11.8 .9 Female (%) 19 (100.0%) 17 (94.4%) 21 (100.0%) .3 Age at disease onset N/A 18–56
33.4 ± 10.9 13–65
32.7 ± 13.5 .8 Disease duration (years) N/A 3–19
9.4 ± 5.2 2–25
10.5 ± 6.8 1.0 SLEDAI N/A 0–8
2.1 ± 2.5 1–25
11.3 ± 6.2 <.01
HC, healthy controls; NPSLE, neuropsychiatric systemic lupus erythematosus; SD, standard deviation; SLE, systemic lupus erythematosus; SLEDAI, systemic lupus erythematosus disease activity index.
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MR Imaging and MR Perfusion
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Imaging Postprocessing and Analysis
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Subjects’ Clinical Assessment
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Table 2
Clinical Components of the SLEDAI, Tallied for the Final Score
Symptom SLEDAI Score Seizure 8 Psychosis 8 Organic brain syndrome 8 Visual disturbance 8 Cranial nerve disorder 8 Lupus headache 8 Stroke 8 Vasculitis 8 Arthritis 4 Myositis 4 Urinary casts 4 Hematuria 4 Proteinuria 4 Pyuria 4 New rash 2 Alopecia 2 Mucosal ulcers 2 Pleurisy 2 Pericarditis 2 Low complement 2 Increased DNA binding 2 Fever 1 Thrombocytopenia 1 Leukopenia 1
SLEDAI, systemic lupus erythematosus disease activity index.
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Statistical Analysis
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Results
Conventional MR Imaging Findings
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MR Perfusion Findings
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Table 3
Relative Cerebral Blood Flow: SLE versus Healthy Controls
SLE HC_P_ value Mean ± SD Mean ± SD Overall (all brain regions) 1.45 ± 0.40 1.32 ± 0.37 .01 Right posterior thalamus 1.50 ± 0.19 1.35 ± 0.21 .03 Right hypothalamus 1.22 ± 0.22 1.07 ± 0.24 .042 Posterior cingulate gyrus 1.58 ± 0.27 1.42 ± 0.23 .048 Left parahippocampal gyrus 1.78 ± 0.30 1.6 ± 0.26 .042
HC, healthy controls; SD, standard deviation; SLE, systemic lupus erythematosus.
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MR Perfusion and Clinical/Laboratory Data
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Discussion
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Conclusion
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