Progressive multifocal leukoencephalopathy immune reconstitution inflammatory syndrome

Last revised by Derek Smith on 7 Jul 2023

Progressive multifocal leukoencephalopathy immune reconstitution inflammatory syndrome (PML-IRIS) is a form of immune reconstitution inflammatory syndrome (IRIS) seen in patients who are immunomodulated and develop an aggressive form of progressive multifocal leukoencephalopathy (PML)

Two types of PML-IRIS are recognized: 

  1. PML-s-IRIS: simultaneous development of IRIS and PML due to immune reconstitution
  2. PML-d-IRIS: immune reconstitution worsens preexisting PML

Both PML-s-IRIS and PML-d-IRIS are encountered in HIV/AIDS 1 and multiple sclerosis, particularly in JC virus positive patients in the setting of prolonged treatment with natalizumab 2

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