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Recent case studies  suggested that pembrolizumab, an anti-PD-1 immune checkpoint inhibitor, may be a potentially efficacious option for  progressive multifocal leukodystrophy (PML) in non-HIV patients. We present a case of a 36-year-old woman who developed PML while undergoing Belimumab therapy. Belimumab, an FDA-approved monoclonal antibody that specifically targets soluble B-lymphocyte stimulators, obtained regulatory approval in 2011 for individuals with systemic lupus erythematosus (SLE). It is worth noting that, to date, only two cases of  PML reported in patients receiving Belimumab.

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 A 36-year-old African American female, diagnosed with systemic lupus erythematosus (SLE) in 2011 and initiated on belimumab therapy in January 2022, presented in September 2023 to an outside hospital with a two-month history of gradually progressive aphasia and right-sided weakness. An MRI revealed a lesion in the left frontal lobe, prompting the administration of intravenous methylprednisone for five days, followed by an oral prednisone taper. She was discharged home as reported plateauing in the progression of symptoms was observed.
However, the patient's condition continued to deteriorate at home, prompting her to seek evaluation at our institution. A repeat of contrast-enhanced MRI of the brain revealed the persistence of the previous lesion in the left frontal lobe and opercular encephalomalacia without gadolinium enhancement. Uncertain as to whether the recent administration of steroids has induced any modifications in the MRI findings. Cerebrospinal fluid (CSF) analysis was benign. Viral screening and cytology were conducted, and JC virus polymerase chain reaction (PCR) was sent from the CSF. Cytology revealed a small population of abnormal B cells. The positive CSF JC virus confirmed the diagnosis of progressive multifocal leukodystrophy (PML). Following the diagnosis, belimumab was discontinued, and the patient was initiated on Pembrolizumab. The patient disease has stabilized and the JCV  CSF titer went remarkably down. 

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