Progressive multifocal leukoencephalopathy immune reconstitution inflammatory syndrome

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:

  • PML-s-IRIS: simultaneous development of IRIS and PML due to immune reconstitution
  • PML-d-IRIS: immune reconstitution worsens preexisting PML
  • Both PML-s-IRIS and PML-d-IRIS are encountered in HIV/AIDS and multiple sclerosis, particularly in JC virus positive patients in the setting of prolonged treatment with natalizumab .