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Unusual Clinical Course in a Case with Relapsing Polychondritis Showing Hypertrophic Pachymeningitis Complicated by a
Ken Yasuda1, Takakuni Maki1, Kohei Nishimura1
1Department of Neurology, Graduate School of Medicine, Kyoto University, Japan.
Abstract:
Relapsing polychondritis (RP) is a rare autoimmune disease that may involve the central nervous system. We herein present the first reported case of hypertrophic pachymeningitis (HP) in an RP patient with a concurrent Klebsiella pneumoniae infection. A 48-year-old man developed transient left-sided weakness and numbness, and brain MRI showed diffuse pachymeningitis. Cultures from a dural biopsy identified K. pneumoniae, and RP was diagnosed using an auricular cartilage biopsy. Treatment with corticosteroids and infliximab resulted in symptom resolution. This case highlights the importance of considering bacterial infections in HP associated with RP, while also providing insight into its pathophysiology.
Insights
This case report describes hypertrophic pachymeningitis (HP) in a relapsing polychondritis (RP) patient with a Klebsiella pneumoniae infection. Prompt diagnosis and treatment led to symptom resolution, emphasizing infection
Area of Science:
- Neurology
- Rheumatology
- Infectious Diseases
Background:
- Relapsing polychondritis (RP) is a rare autoimmune disorder.
- Central nervous system involvement in RP is uncommon but possible.
- Hypertrophic pachymeningitis (HP) is a rare manifestation.
Purpose of the Study:
- To report the first case of HP in an RP patient with a concurrent Klebsiella pneumoniae infection.
- To highlight the diagnostic and therapeutic considerations for this rare presentation.
Main Methods:
- Case presentation of a 48-year-old male with neurological symptoms.
- Brain MRI to evaluate pachymeningitis.
- Dural biopsy for microbial identification.
- Auricular cartilage biopsy for RP diagnosis.
- Treatment with corticosteroids and infliximab.
Main Results:
- The patient presented with transient neurological deficits.
- Brain MRI revealed diffuse pachymeningitis.
- Klebsiella pneumoniae was identified from a dural biopsy.
- RP was confirmed via auricular cartilage biopsy.
- Symptoms resolved following treatment.
Conclusions:
- This case underscores the importance of considering bacterial infections in patients with RP and HP.
- Concurrent Klebsiella pneumoniae infection can mimic or exacerbate HP in RP.
- Integrated management of autoimmune disease and infection is crucial for favorable outcomes.
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