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PML and rheumatology: the contribution of disease and drugs

Eamonn S Molloy1

  • 1Department of Rheumatology, St. Vincent's University Hospital, Elm Park, Dublin 4, Ireland. E.Molloy@st-vincents.ie

Cleveland Clinic Journal of Medicine
|November 30, 2011
PubMed

Insights

Progressive multifocal leukoencephalopathy (PML) is a rare JC virus infection linked to immunosuppressive therapies for autoimmune disorders. Patients with rheumatic diseases, particularly systemic lupus erythematosus, face PML risks, necessitating careful diagnosis and management.

Area of Science:

  • Neurology
  • Immunology
  • Infectious Diseases

Background:

  • Progressive multifocal leukoencephalopathy (PML) is a rare, fatal opportunistic infection caused by the JC virus.
  • PML is increasingly relevant to physicians treating autoimmune disorders with biologic agents.
  • Regulatory alerts highlight PML risks associated with natalizumab, rituximab, efalizumab, and mycophenolate mofetil.

Purpose of the Study:

  • To assess the relative frequency of PML in patients with rheumatic conditions.
  • To inform clinical decision-making regarding PML risk with immunosuppressive therapies.
  • To emphasize the importance of considering PML in the differential diagnosis of neurologic symptoms in rheumatic diseases.

Main Methods:

  • Analysis of PML occurrence in patients with rheumatic diseases.
  • Evaluation of PML risk factors, including specific rheumatic conditions and immunosuppressive therapies.
  • Review of current understanding of PML clinical presentation and diagnosis.

Main Results:

  • Systemic lupus erythematosus (SLE) shows a susceptibility to PML not solely explained by immunosuppression intensity.
  • Rituximab use in rheumatic disease patients raises concerns, though definitive causal attribution is limited by data.
  • All rheumatic disease patients, irrespective of immunosuppression intensity, are potentially at risk for PML.

Conclusions:

  • PML is a significant concern in patients with rheumatic diseases, especially those on immunosuppressive agents.
  • The clinical presentation of PML is heterogeneous, and diagnostic methods are advancing.
  • PML should be a key consideration in the differential diagnosis of neurological manifestations in patients with rheumatic diseases.

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