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Relapsing Polychondritis and Aseptic Meningoencephalitis

Kazuhiro Yokota1, Hideyuki Tachibana2, Akifumi Miyake3

  • 1Department of Rheumatology and Applied Immunology, Faculty of Medicine, Saitama Medical University, Japan.

Internal Medicine (Tokyo, Japan)
|July 13, 2022
PubMed

Insights

Relapsing polychondritis (RP) can rarely cause aseptic meningoencephalitis. Prompt, aggressive immunosuppressive therapy, including steroids and cyclophosphamide, significantly improved cognitive impairment in a patient with RP.

Area of Science:

  • Neurology
  • Immunology
  • Rheumatology

Background:

  • Relapsing polychondritis (RP) is a rare systemic autoimmune disease primarily affecting cartilage.
  • Neurological manifestations of RP are uncommon but can be severe.

Observation:

  • A 49-year-old man with a history of RP developed progressive cognitive impairment.
  • The patient's neurological symptoms emerged despite initial treatment for RP.

Findings:

  • Aggressive immunosuppressive therapy, including methylprednisolone pulse therapy, oral prednisolone (PSL), and intravenous cyclophosphamide, was initiated.
  • The combination therapy led to a clear improvement in the patient's cognitive impairment.

Implications:

  • This case underscores the rare but serious neurological complication of aseptic meningoencephalitis in relapsing polychondritis.
  • Early and intensive immunosuppression is crucial for managing neurological involvement in RP.
Keywords:
aseptic meningoencephalitisglucocorticoidsimmunosuppressantsinterleukin-6relapsing polychondritis

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