IMAGING INSIGHTS INTO JC VIRUS-ASSOCIATED PROGRESSIVE MULTIFOCAL LEUKOENCEPHALOPATHY IN NEUROPSYCHIATRIC LUPUS: A CASE REPORT

PV283 / #106 Case Report Poster Topic: AS05 - CNS Lupus Introduction Progressive multifocal leukoencephalopathy (PML) is a rare, often fatal demyelinating disease of the central nervous system caused by JC virus reactivation in immunocompromised patients.[1] PML is particularly challenging in patients with systemic lupus erythematosus (SLE), where the differential diagnosis between PML and neuropsychiatric lupus (NPSLE) can be difficult, especially when complicated by the use of immunosuppressive therapies like rituximab. Case Presentation With Investigation We present a 40-year-old female diagnosed with SLE and class IV lupus nephritis, managed with hydroxychloroquine 200 mg/day, mycophenolate mofetil 500 mg/day, and prednisolone 10 mg/week, presented with right hemiparesis. Neurological examination revealed muscle power scores of 3 in the right limbs and 5 on the left side. She was diagnosed with an SLE flare-up and left-predominant brain vasculitis, resulting in neuropsychiatric SLE with right hemiplegia. Pulse steroid therapy combined with Cyclosporine 100mg/day was initiated. However, her right limb muscle power declined to 0 after 1 month. Subsequently, pulse steroid therapy, plasma exchange, IVIG, and rituximab (total accumulated dose of 1000mg) were administered in the following month. However, 1 month following rituximab infusion, she exhibited cognitive dysfunction, including dyscalculia, apraxia, and dysphagia. Over the subsequent 2 months, her conscious level gradually declined to E2V1M2, accompanied with quadriparesis. Contrast-enhanced MRI revealed larger T2-hyperintense areas involving bilateral cerebral white matter, basal ganglia, thalami, left midbrain, and left cerebellum (Figure 1). Electroencephalography demonstrated continuously diffuse theta to delta slow waves over bilateral hemispheres without epileptiform discharges or spike waves, indicative of mild generalized encephalopathy. A lumbar puncture was performed, and cerebrospinal fluid examination identified JC virus by PCR method. Now she is under IVIG/3 weeks, and immunosuppressive medications is tapered gradually. Additionally, mefloquine with mirtazapine was initiated after family consultation. Figure 1. MRI progression in a 40-year-old female with SLE and CNS vasculitis evolving into JC virus-related progressive multifocal leukoencephalopathy (PML). Serial axial MRI images from 2023/08 to 2024/01 demonstrate progressive white matter changes. Literature Review JC virus-related PML is a rare and often fatal disease primarily seen in immunocompromised patients.[1] Iatrogenic PML has been associated with rituximab use in lymphoproliferative disorders and occasionally in SLE.[2-3] MRI typically shows hyperintense lesions on T2-weighted FLAIR involving subcortical and juxtacortical white matter.[1] Immune reconstitution is the main treatment strategy due to limited efficacy of direct antiviral agents.[1-3] Discussion This case highlights the difficulty in differentiating between NPSLE and rituximab-associated PML, both of which can present with similar neurological symptoms and imaging findings. While rituximab has proven effective in treating various autoimmune disorders, its association with PML raises concerns, especially in long-term immunosuppressed SLE patients.[3] This case underlines the need for vigilance in monitoring such patients, as early recognition and intervention are key to managing PML. Clinicians should consider a careful balance between treating NPSLE and minimizing the risk of JC virus reactivation, especially in high-risk individuals undergoing rituximab therapy. References: [1.] Cortese I. Nat Rev Neurol 2021;17:37-51. [2.] Carson KR. Blood 2009;113:4834-40. [3.] Raisch DW. Expert Opin Drug Saf 2016;15:1003-11.

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