Session Information
Session Title: Systemic Lupus Erythematosus - Clinical Aspects and Treatment: Epidemiology, Women's Health, Cardiovascular and CNS
Session Type: Abstract Submissions (ACR)
Background/Purpose
Little is known about cerebral small vessel disease (CSVD) in SLE, compared to the mounting evidence of relating cardiovascular risk factors and inflammation to lager vessel disease and atherosclerosis. Our aim is to examine the clinical features of CSVD in SLE, and determine the risk factors associated with CSVD.
Methods
This is a postmortem study of consecutive autopsy brain of subjects drawn from the Maryland Lupus Cohort, between 2002 and 2014. A total of 15 eligible patients were identified, and central nervous system and systemic autopsy histopathology were reviewed. Four patients with limited autopsy data were excluded. Histopathological evidence of CSVD included microthromboemoli, glial hyperplasia, neuronal loss, microaneuryms, lacunar infarcts, and microbleeds. Demographics, clinical SLE features, cardiovascular risk factors, neuroimaging findings and therapeutic options were examined among those with CSVD and those with no CSVD. Multivariate analysis and non parametric studies were used to determine factors associated with CSVD. SLE disease activity (SLEDAI-2k), SLE damage index (SDI) and duration of SLE were adjusted for during analyses.
Results
Only four out of 11 SLE patients (36.4 %) had histopathological evidence of CSVD [n=4, mean age = 26.4 +/- 9.2 years, 100% women, 100% African American, and mean duration of disease = 3.4 +/- 0.9 years] as compared to SLE patients with no CSVD [n=7, mean age = 26.2 +/- 8.8 years, 86 % women, 86 % African American, and mean duration of disease = 3.7 +/- 1.7 years]. CSVD patients presented with less acute CNS disease process and tended to accumulate more damage overtime as compared with no CSVD patients. Stroke and cognitive impairment were more frequent among CSVD patients compared to no CSVD group.
Histopathological CSVD findings correlated well with neuroimaging evidence of CSVD including, the presence of recent subcortical infarcts, lacunae of vascular origin, white matter hyperintensity, and brain atrophy (Pearson correlation coefficient: 0.633; p value < 0.036). CSVD was manifested largely as microinfarction in the subcortical and cortical areas of the frontal and parietal regions (75 %), with volume loss (50 %), non inflammatory vasculopathy (50 %), and inflammatory vessel disease (25 %).
Independent risk factors associated with the occurrence of CSVD included, elevated dsDNA and low levels of C3 (odds ratio; 2.0; 95 % CI: 0.8-5.6, p value < 0.050). This association was independent of age, hypertension, diabetes, smoking, alcohol intake, anti-phospholipid antibodies, C-reactive protein, prior use of immunosuppressive therapy, or presence of microscopic evidence of small vessel disease in other organs such as the kidney, skin, cardiac, or pulmonary systems.
Conclusion
The present study highlights the nature of CSVD in SLE which is of considerable importance related to stroke and cognitive impairment. CSVD in SLE appears to be associated with chronic inflammation in the absence of classic cardiovascular risk factors, or effective treatment of hypertension and SLE. Identifying novel risk factors that shed light on CSVD pathogenesis in SLE may offer potential therapeutic targets.
Disclosure:
J. A. Mikdashi,
None;
R. I. Mehta,
None;
R. J. Castellani,
None.
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ACR Meeting Abstracts - http://acrabstracts.org/abstract/cerebral-small-vessel-disease-in-systemic-lupus-erythematosus-histopathological-study/