MRI in SLE
Magnetic Resonance Imaging and Brain Histopathology in Neuropsychiatric Systemic Lupus Erythematosus

https://doi.org/10.1016/j.semarthrit.2009.08.005Get rights and content

Objective

Magnetic resonance imaging (MRI) often demonstrates brain lesions in neuropsychiatric systemic lupus erythematosus (NPSLE). The present study compared postmortem histopathology with premortem MRI in NPSLE.

Methods

Two hundred subjects with NPSLE were studied prospectively with MRI over a 10-year period during which 22 subjects died. In 14 subjects, a brain autopsy with histopathology, that permitted direct comparison with premortem MRI, was successfully obtained. Surface anatomy was used to determine the approximate location of individual lesions.

Results

Premortem MRI findings in fatal NPSLE were small focal white matter lesions (100%), cortical atrophy (64%), ventricular dilation (57%), cerebral edema (50%), diffuse white matter abnormalities (43%), focal atrophy (36%), cerebral infarction (29%), acute leukoencephalopathy (25%), intracranial hemorrhage (21%), and calcifications (7%). Microscopic findings in fatal NPSLE included global ischemic changes (57%), parenchymal edema (50%), microhemorrhages (43%), glial hyperplasia (43%), diffuse neuronal/axonal loss (36%), resolved cerebral infarction (33%), microthomboemboli (29%), blood vessel remodeling (29%), acute cerebral infarction (14%), acute macrohemorrhages (14%), and resolved intracranial hemorrhages (7%). Cortical atrophy and ventricular dilation seen by MRI accurately predicted brain mass at autopsy (r = −0.72, P = 0.01, and r = −0.77, P = 0.01, respectively). Cerebral autopsy findings, including infarction, cerebral edema, intracranial hemorrhage, calcifications, cysts, and focal atrophy, were also predicted accurately by premortem MRI.

Conclusion

Brain lesions in NPSLE detected by MRI accurately represent serious underlying cerebrovascular and parenchymal brain injury on pathology.

Section snippets

Study Design

This study was approved by the institutional review board and complied with the Declaration of Helinski. Each participant provided a priori written informed consent for both the clinical studies and the postmortem autopsy. The diagnosis of systemic lupus erythematosus (SLE) was established in each subject using the American Rheumatism Association 1982 and American College of Rheumatology (ACR) 1997 revised criteria for SLE (17, 18). A rheumatologist confirmed the diagnosis of SLE after an

Results

Summary demographic and clinical data are included in Table 1 and SLE-related antibody data are included in Table 2. MRI and histopathologic findings are summarized in Table 3. Examples of paired MRI and histopathologic findings are shown in Figure 1, Figure 2, Figure 3, Figure 4, Figure 5, Figure 6, Figure 7, Figure 8, Figure 9. The most common premortem MRI findings in fatal NPSLE were small focal white matter lesions (seen in 100% of subjects), moderate to severe cortical atrophy (64%),

Discussion

There are a number of prior paired MRI-autopsy case reports and a few classic SLE autopsy series (26, 27, 28, 29, 30, 31, 32, 33, 34, 35, 36, 37, 38, 39, 40, 41, 42). However, this is the first prospective study of NPSLE to systematically examine the histopathologic basis for the MRI findings in NPSLE by comparing premortem MRI with histopathology at autopsy (Table 3, Table 4).

The present study demonstrates that thromboembolism and hypercoagulability are dominant mechanisms for fatal NPSLE and

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    This work was supported by research grants from the US National Institutes of Health including R01 HL077422-01-A3 to Dr. Roldan, R01 NS039123 to Dr. Brooks, and R01 NS035708 to Dr. Sibbitt.

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