Intrathecal activation of CD8
CSF single-cell sequencing
IgG4-related disease
cytotoxic T helper cell
inflammatory pseudotumor
pathogenic B-cell
Journal
EMBO molecular medicine
ISSN: 1757-4684
Titre abrégé: EMBO Mol Med
Pays: England
ID NLM: 101487380
Informations de publication
Date de publication:
09 08 2021
09 08 2021
Historique:
revised:
03
06
2021
received:
13
01
2021
accepted:
04
06
2021
pubmed:
14
7
2021
medline:
26
10
2021
entrez:
13
7
2021
Statut:
ppublish
Résumé
IgG4-related disease (IgG4-RD) is a fibroinflammatory disorder signified by aberrant infiltration of IgG4-restricted plasma cells into a variety of organs. Clinical presentation is heterogeneous, and pathophysiological mechanisms of IgG4-RD remain elusive. There are very few cases of IgG4-RD with isolated central nervous system manifestation. By leveraging single-cell sequencing of the cerebrospinal fluid (CSF) of a patient with an inflammatory intracranial pseudotumor, we provide novel insights into the immunopathophysiology of IgG4-RD. Our data illustrate an IgG4-RD-associated polyclonal T-cell response in the CSF and an oligoclonal T-cell response in the parenchymal lesions, the latter being the result of a multifaceted cell-cell interaction between immune cell subsets and pathogenic B cells. We demonstrate that CD8
Identifiants
pubmed: 34254741
doi: 10.15252/emmm.202113953
pmc: PMC8350898
doi:
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
e13953Informations de copyright
© 2021 The Authors. Published under the terms of the CC BY 4.0 license.
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