期刊
JOURNAL OF INVESTIGATIVE MEDICINE HIGH IMPACT CASE REPORTS
卷 5, 期 3, 页码 -出版社
SAGE PUBLICATIONS LTD
DOI: 10.1177/2324709617734248
关键词
progressive multifocal leukoencephalopathy; multiple sclerosis; inflammation; natalizumab
资金
- NIH NIAID Autoimmune Center of Excellence [UM1-AI110557]
- NIH NINDS [R01-NS080821]
- Chugai
- Kirschstein-NRSA [4T32HD007505-20]
- Novartis
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.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据