J Neurocrit Care.  2021 Dec;14(2):103-108. 10.18700/jnc.210028.

Successful treatment with rituximab in a patient with lupus cerebritis and posterior reversible encephalopathy syndrome: a case report

• Choi S
• Kim YS

• KMID: 2523720
• DOI: http://doi.org/10.18700/jnc.210028

Abstract

Background
Neuropsychiatric systemic lupus erythematosus (NPSLE) has a diverse and broad spectrum of severity and prognosis, with some devastating manifestations. However, its diagnosis and treatment remain unclear and controversial.
Case Report
A 19-year-old woman with SLE presented with fever, headache, quadriparesis, and tremor. Brain magnetic resonance imaging (MRI) showed sulcal enhancement in the cortical sulcus, and intravenous methylprednisolone (500 mg/day) and immunoglobulin (2 g/kg for 5 days) were started under the suspicion of aseptic lupus meningitis. However, the patient’s neurologic symptoms worsened; brain MRI showed a newly developed brain parenchymal lesion, suggesting lupus cerebritis and posterior reversible encephalopathy syndrome. Two cycles of rituximab (850 mg/day, 1-week interval) were administered for the treatment of refractory NPSLE. Her neurologic symptoms gradually improved after the second cycle, and she was discharged with minimal neurologic symptoms.
Conclusion
Rituximab may be a therapeutic option for refractory lupus cerebritis. Further research is needed to accurately determine its efficacy.

Keyword

Neuropsychiatric systemic lupus erythematosus; Lupus cerebritis; Posterior reversible encephalopathy syndrome; Rituximab

Figure

• 

  Fig. 1. Initial brain magnetic resonance imaging. (A) Fluid-attenuated inversion recovery (FLAIR) image revealed no abnormal signal intensities. (B) FLAIR-enhanced image revealed multifocal sulcal enhancement in the cortical sulcus, suggesting lupus meningitis.

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  Fig. 2. Brain magnetic resonance imaging after intravenous methylprednisolone and immunoglobulin treatment. Fluid-attenuated inversion recovery (FLAIR) image revealed possible posterior reversible encephalopathy syndrome (white arrows) and possible lupus cerebritis in the right basal ganglia region (red arrow). Diffusion weighted imaging (DWI), susceptibility-weighted imaging (SWI), and time-of-flight magnetic resonance angiography (MRA) showed no evidence of ischemic and hemorrhagic vascular lesions.

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  Fig. 3. Clinical course of the patient. MRC, Medical Research Council; HD, hospital day; IV, intravenous; mPDS, methylprednisolone.

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  Fig. 4. Brain magnetic resonance imaging performed 3 months after rituximab treatment. Fluid-attenuated inversion recovery (FLAIR) image (A) and FLAIR-enhanced image (B) showed no abnormalities.

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