PMID- 28989935
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20191120
IS  - 2324-7096 (Print)
IS  - 2324-7096 (Electronic)
IS  - 2324-7096 (Linking)
VI  - 5
IP  - 3
DP  - 2017 Jul-Sep
TI  - Pathologic Findings of Chronic PML-IRIS in a Patient with Prolonged PML Survival 
      Following Natalizumab Treatment.
PG  - 2324709617734248
LID - 10.1177/2324709617734248 [doi]
LID - 2324709617734248
AB  - Immune reconstitution inflammatory syndrome (IRIS) is a common complication 
      during treatment for natalizumab-associated progressive multifocal 
      leukoencephalopathy (PML). Although severe IRIS can result in acute worsening of 
      disability and is associated with poor prognosis, effective immune reconstitution 
      may account for the high survival rate of this cohort of PML patients. We present 
      pathological evidence of chronic IRIS 3.5 years after diagnosis with 
      natalizumab-associated PML. Our case showed that the IRIS initially developed 
      after plasma exchange therapy and resolved clinically and radiologically 
      following a combination treatment with corticosteroids, maraviroc, and cidofovir. 
      Autopsy 3.5 years later revealed evidence of grey-white matter junction 
      demyelinating lesions characteristic of PML and perivascular leukocyte 
      infiltrates predominated by CD8(+) T-lymphocytes, and polymerase chain reaction 
      analysis demonstrated the presence of JC viral DNA in this tissue, indicative of 
      persistent PML-IRIS. While clinical symptoms of PML-IRIS typically stabilize 
      within 6 months, our case report suggests that prolonged low-grade inflammation 
      may persist in some patients. Better assays are needed to determine the 
      prevalence of prolonged low-grade IRIS among PML survivors.
FAU - Himedan, Mai
AU  - Himedan M
AD  - University of Michigan, Ann Arbor, MI, USA.
FAU - Camelo-Piragua, Sandra
AU  - Camelo-Piragua S
AD  - University of Michigan, Ann Arbor, MI, USA.
FAU - Mills, Elizabeth A
AU  - Mills EA
AD  - University of Michigan, Ann Arbor, MI, USA.
FAU - Gupta, Avneesh
AU  - Gupta A
AD  - University of Michigan, Ann Arbor, MI, USA.
FAU - Aburashed, Rany
AU  - Aburashed R
AD  - Michigan State University, East Lansing, MI, USA.
FAU - Mao-Draayer, Yang
AU  - Mao-Draayer Y
AD  - University of Michigan, Ann Arbor, MI, USA.
LA  - eng
GR  - R01 NS080821/NS/NINDS NIH HHS/United States
GR  - T32 HD007505/HD/NICHD NIH HHS/United States
GR  - UM1 AI110557/AI/NIAID NIH HHS/United States
PT  - Journal Article
DEP - 20170927
PL  - United States
TA  - J Investig Med High Impact Case Rep
JT  - Journal of investigative medicine high impact case reports
JID - 101624758
PMC - PMC5624358
OTO - NOTNLM
OT  - inflammation
OT  - multiple sclerosis
OT  - natalizumab
OT  - progressive multifocal leukoencephalopathy
COIS- Declaration of Conflicting Interests: The author(s) declared the following 
      potential conflicts of interest with respect to the research, authorship, and/or 
      publication of this article: YMD has served as a consultant and/or received grant 
      support from Acorda, Bayer Pharmaceutical, EMD Serono, Genzyme, Novartis, 
      Questor, Teva Neuroscience, and Chugai Pharma. RA has served as a 
      consultant/advisor and faculty for Sanofi-Genzyme, Bayer, Biogen, Genentech, and 
      Novartis; has served on speaker bureau for noncommercial and commercial 
      engagements for Sanofi Genzyme, Teva, Novartis, Genentech, and Biogen. RA also 
      received research grants from Biogen and Novartis. MH, SCP, EAM, and AG have no 
      conflicts of interest.
EDAT- 2017/10/11 06:00
MHDA- 2017/10/11 06:01
PMCR- 2017/09/27
CRDT- 2017/10/10 06:00
PHST- 2017/08/10 00:00 [received]
PHST- 2017/09/01 00:00 [revised]
PHST- 2017/09/04 00:00 [accepted]
PHST- 2017/10/10 06:00 [entrez]
PHST- 2017/10/11 06:00 [pubmed]
PHST- 2017/10/11 06:01 [medline]
PHST- 2017/09/27 00:00 [pmc-release]
AID - 10.1177_2324709617734248 [pii]
AID - 10.1177/2324709617734248 [doi]
PST - epublish
SO  - J Investig Med High Impact Case Rep. 2017 Sep 27;5(3):2324709617734248. doi: 
      10.1177/2324709617734248. eCollection 2017 Jul-Sep.