Abstract
Background:
The cytotoxic T-lymphocyte antigen-4 (CTLA-4) pathway acts as a negative immune regulator of T cell activation and promotes self-tolerance.
Case:
We report the first case of biopsy-proven central nervous system inflammatory demyelination in the context of primary immunodeficiency and a novel CTLA-4 variant.
Conclusions:
This case has significant implications for the development of novel treatments for autoimmune conditions including Multiple Sclerosis and further emphasises the need for caution with clinical use of CTLA-4 immune checkpoint inhibitors in those with a history of inflammatory demyelination.
The cytotoxic T-lymphocyte antigen-4 (CTLA-4) pathway acts as a negative immune regulator of T cell activation and promotes self-tolerance.
Case:
We report the first case of biopsy-proven central nervous system inflammatory demyelination in the context of primary immunodeficiency and a novel CTLA-4 variant.
Conclusions:
This case has significant implications for the development of novel treatments for autoimmune conditions including Multiple Sclerosis and further emphasises the need for caution with clinical use of CTLA-4 immune checkpoint inhibitors in those with a history of inflammatory demyelination.
| Original language | English |
|---|---|
| Pages (from-to) | 1464-1467 |
| Number of pages | 4 |
| Journal | Multiple Sclerosis Journal |
| Volume | 27 |
| Issue number | 9 |
| Early online date | 7 Jun 2021 |
| DOIs | |
| Publication status | Published - Aug 2021 |
Bibliographical note
Funding Information:The author(s) disclosed receipt of the following financial support for the research, authorship and/or publication of this article: No targeted funding reported. S.K. is supported by an ECTRIMS (European Committee for Treatment and Research in Multiple Sclerosis) fellowship.
Publisher Copyright:
© The Author(s), 2021.
Keywords
- CTLA-4
- demyelination