Ear and Nose Abnormalities in Meningoencephalitis Associated With Relapsing Polychondritis: A Case Report
Tomoaki Taguchi1, Soichiro Matsubara1, Keiichi Nakahara1
1Neurology, Kumamoto University, Kumamoto, JPN.
Cureus
|July 4, 2025
Summary
Relapsing polychondritis (RPC) can cause meningoencephalitis, a challenging diagnosis. Early recognition of ear and nose deformities aids in identifying this rare autoimmune condition with central nervous system involvement.
Area of Science:
- Rheumatology
- Neurology
- Pathology
Background:
- Relapsing polychondritis (RPC) is a rare autoimmune disorder primarily affecting cartilage and connective tissues.
- RPC diagnosis is challenging due to its low incidence and varied presentations.
- Meningoencephalitis as the dominant symptom of RPC is particularly difficult to diagnose.
Observation:
- A 67-year-old woman presented with fever, impaired consciousness, and nuchal rigidity, initially diagnosed as aseptic meningoencephalitis.
- Cerebrospinal fluid analysis showed pleocytosis and elevated Interleukin-6 (IL-6).
- Head MRI revealed meningeal enhancement, and physical examination noted ear and nose deformities.
Findings:
- Histopathological examination of a pinna biopsy confirmed relapsing polychondritis.
- The patient was diagnosed with RPC involving the central nervous system (CNS).
- Immunotherapy led to partial symptom relief and decreased intrathecal IL-6 levels.
Implications:
- Auricular chondritis (ear inflammation) in RPC can serve as a crucial diagnostic clue.
- Neurologists should consider RPC in cases of meningoencephalitis of unknown origin.
- Prompt recognition of characteristic physical abnormalities can facilitate timely diagnosis and treatment of RPC with CNS involvement.
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