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CLIPPERS Responsive to Cladribine as a Durable Steroid-Sparing Agent

OBJECTIVE: We report a case of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) who achieved durable and steroid-free remission after IV cladribine. METHODS: A 25 year-old man presented with progressively worsening headaches, polydipsia, dysart...

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Autores principales: Thebault, Simon, Bergman, Hailey, Atkins, Harold L., Freedman, Mark S., Brooks, John
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Lippincott Williams & Wilkins 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9747139/
https://www.ncbi.nlm.nih.gov/pubmed/36396449
http://dx.doi.org/10.1212/NXI.0000000000200060
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author Thebault, Simon
Bergman, Hailey
Atkins, Harold L.
Freedman, Mark S.
Brooks, John
author_facet Thebault, Simon
Bergman, Hailey
Atkins, Harold L.
Freedman, Mark S.
Brooks, John
author_sort Thebault, Simon
collection PubMed
description OBJECTIVE: We report a case of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) who achieved durable and steroid-free remission after IV cladribine. METHODS: A 25 year-old man presented with progressively worsening headaches, polydipsia, dysarthria, diplopia and vertigo, and obtundation requiring respiratory support. CSF revealed lymphocytosis, and MRI revealed a perivascular pattern of punctate enhancement involving the pons. An extensive workup for inflammatory, autoimmune, infective, and malignant explanations was unrevealing. He responded dramatically to steroids, compatible with CLIPPERS as a diagnosis of exclusion. Attempts to wean prednisone over the ensuing year resulted in 2 clinical relapses and persistent punctate enhancement. Given significant steroid side effects, steroid-sparing agents were considered. RESULTS: IV cladribine IV (0.0875 mg/kg adjusted body weight daily × 4 days at 0, 4, 8, and 16 months) was selected, given its favorable side effect profile including lower risks of malignancy and infertility and the potential for long-lasting effects. The only side effect was short-term fatigue at the time of infusion. At 20 months after cladribine initiation, he was able to wean-off prednisone altogether. Now at 33 months, he remains in clinical and MRI remission. DISCUSSION: Cladribine is a rational candidate steroid-sparing treatment for presumed neurologic autoimmune conditions such as CLIPPERS. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that cladribine is a steroid-sparing treatment consideration in CLIPPERS.
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spelling pubmed-97471392022-12-14 CLIPPERS Responsive to Cladribine as a Durable Steroid-Sparing Agent Thebault, Simon Bergman, Hailey Atkins, Harold L. Freedman, Mark S. Brooks, John Neurol Neuroimmunol Neuroinflamm Clinical/Scientific Note OBJECTIVE: We report a case of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) who achieved durable and steroid-free remission after IV cladribine. METHODS: A 25 year-old man presented with progressively worsening headaches, polydipsia, dysarthria, diplopia and vertigo, and obtundation requiring respiratory support. CSF revealed lymphocytosis, and MRI revealed a perivascular pattern of punctate enhancement involving the pons. An extensive workup for inflammatory, autoimmune, infective, and malignant explanations was unrevealing. He responded dramatically to steroids, compatible with CLIPPERS as a diagnosis of exclusion. Attempts to wean prednisone over the ensuing year resulted in 2 clinical relapses and persistent punctate enhancement. Given significant steroid side effects, steroid-sparing agents were considered. RESULTS: IV cladribine IV (0.0875 mg/kg adjusted body weight daily × 4 days at 0, 4, 8, and 16 months) was selected, given its favorable side effect profile including lower risks of malignancy and infertility and the potential for long-lasting effects. The only side effect was short-term fatigue at the time of infusion. At 20 months after cladribine initiation, he was able to wean-off prednisone altogether. Now at 33 months, he remains in clinical and MRI remission. DISCUSSION: Cladribine is a rational candidate steroid-sparing treatment for presumed neurologic autoimmune conditions such as CLIPPERS. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that cladribine is a steroid-sparing treatment consideration in CLIPPERS. Lippincott Williams & Wilkins 2022-11-17 /pmc/articles/PMC9747139/ /pubmed/36396449 http://dx.doi.org/10.1212/NXI.0000000000200060 Text en Copyright © 2022 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND) (https://creativecommons.org/licenses/by-nc-nd/4.0/) , which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.
spellingShingle Clinical/Scientific Note
Thebault, Simon
Bergman, Hailey
Atkins, Harold L.
Freedman, Mark S.
Brooks, John
CLIPPERS Responsive to Cladribine as a Durable Steroid-Sparing Agent
title CLIPPERS Responsive to Cladribine as a Durable Steroid-Sparing Agent
title_full CLIPPERS Responsive to Cladribine as a Durable Steroid-Sparing Agent
title_fullStr CLIPPERS Responsive to Cladribine as a Durable Steroid-Sparing Agent
title_full_unstemmed CLIPPERS Responsive to Cladribine as a Durable Steroid-Sparing Agent
title_short CLIPPERS Responsive to Cladribine as a Durable Steroid-Sparing Agent
title_sort clippers responsive to cladribine as a durable steroid-sparing agent
topic Clinical/Scientific Note
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9747139/
https://www.ncbi.nlm.nih.gov/pubmed/36396449
http://dx.doi.org/10.1212/NXI.0000000000200060
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