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Progressive multifocal leukoencephalopathy in patients with rheumatic diseases: are patients with systemic lupus

Eamonn S Molloy1, Leonard H Calabrese

  • 1Department of Rheumatic and Immunologic Diseases, Cleveland Clinic, Cleveland, Ohio 44195, USA. molloye@ccf.org

Autoimmunity Reviews
|August 14, 2008
PubMed

Insights

Progressive multifocal leukoencephalopathy (PML) is a rare, fatal infection in immunocompromised patients. Systemic lupus erythematosus (SLE) patients may have an unexplained predisposition to PML, even with minimal immunosuppression.

Area of Science:

  • Neurology
  • Rheumatology
  • Infectious Diseases

Background:

  • Progressive multifocal leukoencephalopathy (PML) is a rare, fatal opportunistic infection.
  • PML is a known complication in immunocompromised patients, including those with rheumatic diseases on immunosuppressive therapy.
  • Recent cases of PML associated with natalizumab and rituximab highlight the risk in rheumatic disease patients.

Purpose of the Study:

  • To review the occurrence of PML in patients with rheumatic diseases.
  • To investigate the association between systemic lupus erythematosus (SLE) and PML.
  • To explore potential predisposing factors for PML in SLE patients.

Main Methods:

  • Literature review of reported PML cases in patients with rheumatic diseases.
  • Analysis of PML incidence in SLE patients, considering levels of immunosuppression.

Main Results:

  • Nearly two-thirds of reported PML cases in rheumatic diseases occurred in SLE patients.
  • Over 40% of PML cases in SLE patients involved minimal iatrogenic immunosuppression.
  • This suggests SLE itself may predispose patients to PML.

Conclusions:

  • SLE patients appear to have a higher predisposition to PML than other rheumatic disease patients.
  • The underlying mechanism for SLE predisposition to PML remains unclear.
  • Further research is needed to understand the link between SLE and PML development.

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