The Risk of Progressive Multifocal Leukoencephalopathy in the Biologic Era




Progressive multifocal leukoencephalopathy (PML) is a rare, typically fatal, demyelinating central nervous system infection caused by reactivation of the John Cunningham virus that generally occurs in immunosuppressed patients. With an evolving understanding of a greater clinical heterogeneity of PML and significant implications for therapy, PML should be considered in the differential diagnosis of neurologic presentations of rheumatic diseases. Increased awareness of PML among rheumatologists is required, as earlier diagnosis and restoration of immune function may improve the otherwise grim prognosis associated with PML.


Key points








  • Progressive multifocal leukoencephalopathy (PML) is a frequently fatal demyelinating infection of the central nervous system due to reactivation of the ubiquitous John Cunningham polyomavirus in immunosuppressed patients.



  • PML has been reported in patients with rheumatic disease, with a disproportionate occurrence in systemic lupus erythematosus.



  • A variety of rheumatic disease therapies have been associated with PML, with a disproportionate signal for rituximab. In a notable minority of patients, PML occurs in the setting of minimal iatrogenic immunosuppression.



  • Increased awareness of PML is necessary, as early diagnosis and withdrawal of immunosuppressive therapy may improve outcomes.


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Sep 28, 2017 | Posted by in RHEUMATOLOGY | Comments Off on The Risk of Progressive Multifocal Leukoencephalopathy in the Biologic Era

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