A second major category of disease in acute NPSLE is related to hypertension. Hypertension is common in SLE due to renal disease. Patients with acute worsening of hypertension often present with seizures, headache and sometimes depressed mental status. Imaging findings are similar to those of other conditions such as eclampsia that result in an acute rise in blood pressure and, most likely, altered cerebral autoregulation. Typically this includes subcortical and cortical areas of high T2 signal intensity in several locations, usually with a posterior cerebral predominance. Basal ganglia, cerebellum, and brainstem are involved less commonly. Both imaging and clinical manifestations are usually reversible with control of blood pressure, but in more severe cases, permanent damage can result. Figures 3 and 4 present examples of this hypertensive injury to the brain with excellent recovery and fatal outcome, respectively. Figure 5 presents a different consequence of acute hypertension, cerebral hemorrhage. Pathological correlation was obtained in cases illustrated in Figures 4 and 5.

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Figure 3. Acute encephalopathy in the setting of hypertension. A 51-year-old man with SLE and renal hypertension presented with acute generalized tonic-clonic seizures followed by a persistent confusional state. Axial FLAIR images (A, B, C) show extensive areas of edema in the brain, with a posterior subcortical predominance but also involving the pons. The patient was treated with high dose corticosteroids and intravenous cyclophosphamide. Follow-up MR imaging 3 weeks after the first scan shows marked improvement (D, E, F) that was accompanied by a resolution of the acute confusional state.

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Figure 4. Fatal encephalopathy in SLE. This 22-year-old woman with SLE was admitted with increasing confusion and flat affect. Initial MRI demonstrated only cortical atrophy and a few small subcortical white matter lesions in the frontal lobes. After admission, the patient rapidly deteriorated and suffered a generalized tonic-clonic seizure. She progressed to respiratory failure, was intubated, and experienced further seizures. CT obtained shortly after intubation at admission (A, B, C) shows multifocal regions of edema, especially in the posterior cerebral hemispheres. Note that the basilar cisterns are completely effaced (A). The patient died, and an autopsy was performed. Vasculitis was present in multiple organs, including the heart, lungs, liver, spleen, kidneys, and small intestines, but not in the brain. Thrombotic angiopathy in the brain was limited to the left basal ganglia. Ill-defined areas of pallor were present in cerebral white matter, showing rarefaction and swelling of myelinated fibers. The blood vessels either had unremarkable walls or were surrounded by focal hemorrhages and fibrinoid necrosis. Photomicrograph (D, H & E stain) shows ball hemorrhage surrounding a small vessel in white matter. The clinical, imaging, and pathological findings are consistent with a severe case of hypertensive encephalopathy.

Figure 5. Fatal hypertensive hemorrhage. A young woman with SLE presented at 31 weeks gestation with seizures and systolic blood pressure over 200 mm Hg, progressing rapidly to coma. CT (A, B) showed symmetric, severe, bilateral intraventricular and parenchymal hemorrhage. Autopsy confirmed massive cerebral hemorrhage; there was no evidence of cerebral vasculitis on microscopic examination of the brain.