PML-IRIS
Pronunciation: pee-em-el EYE-ris
Also written as: PML-IRIS — Progressive Multifocal Leukoencephalopathy-Immune Reconstitution Inflammatory Syndrome
Immune reconstitution inflammatory syndrome occurring in progressive multifocal leukoencephalopathy patients after immune system recovery.
Full Definition
PML-IRIS (Progressive Multifocal Leukoencephalopathy-Immune Reconstitution Inflammatory Syndrome) is a paradoxical worsening of neurological symptoms that occurs when the immune system recovers in patients with PML. This syndrome typically develops when immunocompromised patients with PML experience immune reconstitution, either spontaneously or due to treatment modification. The recovering immune system mounts an inflammatory response against JC virus-infected brain tissue, leading to increased tissue damage, cerebral edema, and clinical deterioration despite viral control. PML-IRIS presents diagnostic and therapeutic challenges, as corticosteroids may help reduce inflammation but could potentially worsen the underlying viral infection.
Usage
Usage note: Always hyphenate PML-IRIS; spell out on first use in formal documents.
In Context
- "The patient developed PML-IRIS three months after discontinuing natalizumab therapy." — Clinical case report
- "Distinguishing between PML progression and PML-IRIS requires careful clinical and radiological assessment." — Medical review article