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The emergence of progressive multifocal leukoencephalopathy (PML) in rheumatic diseases

Eric J Boren1, Gurtej S Cheema, Stanley M Naguwa

  • 1University of California at Davis School of Medicine, Department of Internal Medicine, Division of Rheumatology, Allergy, and Clinical Immunology, 451 Health Sciences Drive, Suite 6510, Davis, CA 95616, USA.

Journal of Autoimmunity
|January 15, 2008
PubMed

Insights

Progressive multifocal leukoencephalopathy (PML) is a rare, fatal neurological disease caused by JC virus reactivation in immunosuppressed individuals. PML is increasingly seen in multiple sclerosis and rheumatic diseases, highlighting risks with immune-modulating therapies.

Area of Science:

  • Neurology
  • Immunology
  • Virology

Background:

  • Progressive multifocal leukoencephalopathy (PML) is a rare demyelinating disease of the central nervous system.
  • It is caused by JC virus reactivation, typically in profoundly immunosuppressed individuals.
  • PML was an AIDS-defining illness and is now seen in other conditions like multiple sclerosis and rheumatic diseases.

Purpose of the Study:

  • To review the emergence of PML in new patient populations.
  • To discuss the risks associated with current and future immune suppression therapies.
  • To emphasize the need for caution when considering immune-modulating treatments.

Main Methods:

  • Literature review of PML cases.
  • Analysis of PML association with immunosuppressive therapies.
  • Discussion of JC virus reactivation mechanisms.

Main Results:

  • PML is increasingly reported in patients with multiple sclerosis on natalizumab.
  • PML cases are also observed in rheumatic diseases, with or without biologic agents.
  • Immune suppression and manipulation strategies increase the risk of PML.

Conclusions:

  • JC virus reactivation leading to PML is a significant concern with immune-modulating therapies.
  • Careful consideration and risk assessment are crucial when evaluating treatments that suppress the immune system.
  • Despite emerging risks, PML remains a rare complication.

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