Progressive multifocal leukoencephalopathy in systemic lupus erythematosus managed with pembrolizumab: A case report with literature review.
Lupus
; 30(11): 1849-1855, 2021 Oct.
Article
em En
| MEDLINE
| ID: mdl-34325541
ABSTRACT
Progressive multifocal leukoencephalopathy (PML) is one of the rare but lethal infectious complication in patients with SLE, manifesting progressive central nervous demyelination caused by JC virus (JCV). There have been no effective antiviral agents so far; however, immune checkpoint inhibitors (ICI) have been demonstrated as potential treatments by reinvigorating antiviral T-cell activity against JC virus. To date, sixteen PML cases treated with anti-PD-1 have been reported; however, there was no report addressing the use of ICI in patients with concomitant PML and rheumatic disease, possibly due to the concern for possible autoimmune disease flare-up. In addition, treatment outcomes of these ICI-treated cases were heterogeneous. Experiences from these cases suggested that high disease burden, JC viral load in CSF, and severe immunosuppression status at baseline may predict poor response to treatment. Our case, a 62-year-old woman with long-standing SLE, turned out to have a delayed but effective response to prolonged ICI treatment despite of her high JC viral load and immunosuppressed status caused by high-dose steroid and rituximab. To our knowledge, this is the first case report with SLE complicated with PML clinically improved by pembrolizumab treatment without consequent immune related adverse events (irAE). Considering the lethal nature of PML and absence of effective medication, ICI is a reasonable consideration in patients with SLE and progressive PML.
Palavras-chave
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Leucoencefalopatia Multifocal Progressiva
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Anticorpos Monoclonais Humanizados
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Inibidores de Checkpoint Imunológico
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Lúpus Eritematoso Sistêmico
Tipo de estudo:
Diagnostic_studies
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Etiology_studies
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Prognostic_studies
Limite:
Female
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Humans
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Middle aged
Idioma:
En
Revista:
Lupus
Assunto da revista:
REUMATOLOGIA
Ano de publicação:
2021
Tipo de documento:
Article
País de afiliação:
Taiwan