CLIPPERS syndrome responsive to Leflunomide: A case report

Didier, Parada-Garza Juan; Adrián, Miranda-García Luis; Paola, Salvatella-Gutierrez Ana; Figueroa-Sánchez, Mauricio; Cárdenas-Saenz, Omar; Liset, Roque-Villavicencio Yuridia; Luis, Ruiz-Sandoval Jose

doi:10.1016/j.msard.2018.07.048

Cited by 6 publications

(2 citation statements)

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“…Although high quality or controlled studies are lacking, effectiveness has been demonstrated for methotrexate, azathioprine, and cyclophosphamide. 2 Mycophenolate mofetil, rituximab, mitoxantrone, interferon beta-1a, 3 tocilizumab, 4 and leflunomide 5 have also been used.…”

Section: Discussionmentioning

confidence: 99%

CLIPPERS Responsive to Cladribine as a Durable Steroid-Sparing Agent

Thebault

Bergman²,

Atkins³

et al. 2023

Neurol Neuroimmunol Neuroinflamm

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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.

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Section: Discussionmentioning

confidence: 99%

CLIPPERS Responsive to Cladribine as a Durable Steroid-Sparing Agent

Thebault

Bergman²,

Atkins³

et al. 2023

Neurol Neuroimmunol Neuroinflamm

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“…[43] One case reported that although the clinical symptoms and imaging findings had disappeared completely before receiving weekly oral leflunomide (100 mg), there was no recurrence of symptoms during the follow-up of the following year. [44] Leflunomide is an active metabolite of drugs for the treatment of rheumatoid arthritis, inhibits dihydroorotate dehydrogenase, affects pyrimidine metabolism, and regulates T cells. T cells play an important role in the pathogenesis of CLIPPERS.…”

Section: Symptom Remission Period (After Hormone Pulse Therapy)mentioning

confidence: 99%

The pathogenesis hypothesis and research progress of CLIPPERS: A literature review

et al. 2023

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Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is still a rare autoimmune disease in the world. In recent years, there are more and more reports about the clinical manifestations of CLIPPERS, but the specific etiology and pathogenesis are not clear. In this paper, by collating the literature reported in recent years, in the reported effective treatment cases, we found the current hypothesis about the pathogenesis of CLIPPERS. Three pathogenesis hypotheses: organ-specific autoimmunity; virus infection affects autoimmunity; and helper T lymphocyte 17 mediates autoimmunity. Although it is hypothetical, it is expected to further clarify the pathogenesis, evolution characteristics, and treatment of CLIPPERS, so as to provide a reference for further understanding of the disease. In the future, more observations and studies are needed to further verify the feasibility of the hypothesis. This article expands on atypical clinical manifestations and summarizes treatment options. Hope to provide a reference for clinical diagnosis and treatment of CLIPPERS.

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