Background Progressive multifocal leukoencephalopathy (PML) is a devastating opportunistic infection of the central nervous system, caused by JC virus. PML has classically occurred in immunosuppressed patients, however it has attracted the rheumatologists' attention following reports of its association with some biologic agents.
Although extremely rare, drug-associated PML has led to an aggressive risk-mitigation program for natalizumab, the voluntary withdrawal from the market of efalizumab and several papers regarding rituximab and mycophenolate mofetil associated-risk. Also, some rheumatic conditions seem to be associated with susceptibility to PML that cannot be exclusively explained by the immunosuppressive therapy.
It is important to recognize the possibility of PML in rheumatic diseases, because it can mimic central nervous system involvement and the fundamental first step in treating confirmed or suspected PML is discontinuing the immunosuppressive drug.
Objectives To assess the Portuguese rheumatologists' knowledge about PML, attitudes and practices when prescribing an immunosuppressive drug and to identify specific needs for education about this topic.
Methods Cross-sectional descriptive study using an internet-based survey distributed via email to 120 members of the Portuguese Rheumatology Society. Data were collected from December 2013 to January 2014.
The survey included 10 multiple-choice questions to assess general knowledge about PML, risk factors and implied therapies in the rheumatology field, attitudes and practices when prescribing as immunosuppressive drug, self-reported knowledge about PML and specific needs for education.
Results Responses were received from 57 physicians, which correspond to a response rate of approximately 48%. When asked 68% could identify the causative virus of PML, but only 12% identified the correct prevalence of JC virus infection in general population. The majority of the physicians identified correctly the most frequent clinical manifestations of PML and the diagnostic tests of choice.
About half of the physicians identified correctly the disease associated with a significant higher risk of PML, but only 21% could correctly identify rituximab and mycophenolate mofetil as drugs associated with increased risk of PML. The majority (79%) of the rheumatologists reported not discussing the risk of PML with patients when prescribing an implicated therapy and the same percentage reported having none or low knowledge about PML. Finally, the topics considered most important for education were diseases and drugs associated with PML and risk-benefit analysis of immunosuppressive therapies.
Conclusions Portuguese rheumatologists have perceived low knowledge and high educational needs about PML as a possible complication in rheumatic patients. Topics considered priority for education were diseases and drugs associated with PML and risk-benefit analysis of immunosuppressive therapies.
This study was important to evaluate the Portuguese rheumatologists' knowledge about this serious and potentially treatable disease. The findings of the present study indicate a necessary improvement in knowledge about PML among rheumatologists in order to improve its performance in its recognition.
Cleve Clin J Med. 2011 Nov;78 Suppl 2:S28-32.
Joint Bone Spine. 2012 Jul;79(4):351-5.
Disclosure of Interest None declared