CLIPPERS Responsive to Cladribine as a Durable Steroid-Sparing Agent

1Citations
Citations of this article
7Readers
Mendeley users who have this article in their library.

Abstract

ObjectiveWe 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.MethodsA 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.ResultsIV 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.DiscussionCladribine is a rational candidate steroid-sparing treatment for presumed neurologic autoimmune conditions such as CLIPPERS.Classification of EvidenceThis study provides Class IV evidence that cladribine is a steroid-sparing treatment consideration in CLIPPERS.

Register to see more suggestions

Mendeley helps you to discover research relevant for your work.

Already have an account?

Cite

CITATION STYLE

APA

Thebault, S., Bergman, H., Atkins, H. L., Freedman, M. S., & Brooks, J. (2023). CLIPPERS Responsive to Cladribine as a Durable Steroid-Sparing Agent. Neurology: Neuroimmunology and NeuroInflammation, 10(1). https://doi.org/10.1212/NXI.0000000000200060

Readers' Seniority

Tooltip

PhD / Post grad / Masters / Doc 3

75%

Researcher 1

25%

Readers' Discipline

Tooltip

Medicine and Dentistry 2

40%

Nursing and Health Professions 1

20%

Neuroscience 1

20%

Biochemistry, Genetics and Molecular Bi... 1

20%

Article Metrics

Tooltip
Mentions
News Mentions: 1

Save time finding and organizing research with Mendeley

Sign up for free