title: Incidence and prognosis of immune reconstitution inflammatory syndrome in HIV-associated progressive multifocal leucoencephalopathy.
abstract: OBJECTIVE
  Progressive multifocal leucoencephalopathy-associated immune reconstitution inflammatory syndrome (PML-IRIS) is the paradoxical worsening or unmasking of preexisting infection with JC virus attributable to a rapid recovery of the immune system after highly active antiretroviral therapy (HAART) initiation. We investigated the incidence and factors associated with PML-IRIS in HIV-infected patients. We also studied its influence on mortality of PML and the effect of corticosteroid therapy.
  
  
  METHODS
  Single-center retrospective analysis of HIV-infected patients diagnosed with PML from 1996 to 2012 who received HAART.
  
  
  RESULTS
  Among 59 PML patients treated with HAART, 18 (30.51%) developed PML-IRIS (five delayed PML-IRIS, 13 simultaneous PML-IRIS). Patients who developed IRIS had lower CD4 counts prior to treatment (102 vs. 68.5, P < 0.05) and experienced a greater decline in HIV-RNA levels in response to HAART (2.5log vs. 2.95log, P < 0.05). Gadolinium enhancement on MRI was observed in 31.25% of PML-IRIS cases versus 2.56% of PML non-IRIS (P < 0.01). Survival rates were higher in patients with PML-IRIS compared to those with PML non-IRIS. Eight patients received corticosteroids, five of which had a good outcome. Patients who died were severely ill when treatment was initiated whereas patients who survived were treated before major neurological deterioration occurred.
  
  
  CONCLUSIONS
  Nearly one-third of HIV-infected patients with PML develop IRIS after initiating HAART. Patients severely immunocompromised who experience a rapid virological response to HAART have a higher risk for PML-IRIS. There was a trend for lower mortality in patients with IRIS. Early treatment with corticosteroids might be useful.
authors.affiliation: Department of Neurology, Hospital Universitario Ramón y Cajal, Madrid, Spain.
authors.forename: S
authors.initials: S
authors.lastname: Sainz-de-la-Maza
authors.affiliation: Department of Infectious Diseases, Hospital Universitario Ramón y Cajal, Madrid, Spain.
authors.forename: J L
authors.initials: JL
authors.lastname: Casado
authors.affiliation: Department of Infectious Diseases, Hospital Universitario Ramón y Cajal, Madrid, Spain.
authors.forename: M J
authors.initials: MJ
authors.lastname: Pérez-Elías
authors.affiliation: Department of Infectious Diseases, Hospital Universitario Ramón y Cajal, Madrid, Spain.
authors.forename: A
authors.initials: A
authors.lastname: Moreno
authors.affiliation: Department of Infectious Diseases, Hospital Universitario Ramón y Cajal, Madrid, Spain.
authors.forename: C
authors.initials: C
authors.lastname: Quereda
authors.affiliation: Department of Infectious Diseases, Hospital Universitario Ramón y Cajal, Madrid, Spain.
authors.forename: S
authors.initials: S
authors.lastname: Moreno
authors.affiliation: Department of Neurology, Hospital Universitario Ramón y Cajal, Madrid, Spain.
authors.forename: I
authors.initials: I
authors.lastname: Corral
mesh_terms: D000328:Adult; D023241:Antiretroviral Therapy, Highly Active; D001921:Brain / Q000000981:diagnostic imaging; D005260:Female; D015658:HIV Infections / Q000150:complications / Q000188:drug therapy / Q000453:epidemiology*; D006801:Humans; D054019:Immune Reconstitution Inflammatory Syndrome / Q000000981:diagnostic imaging / Q000453:epidemiology*; D015994:Incidence; D007577:JC Virus / Q000276:immunology; D007968:Leukoencephalopathy, Progressive Multifocal / Q000000981:diagnostic imaging / Q000453:epidemiology* / Q000209:etiology; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies
keywords: AIDS; HIV; JC virus; antiretroviral therapy; immune reconstitution inflammatory syndrome; progressive multifocal leucoencephalopathy