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Relapsing polychondritis: a course over 20 years with cerebral involvement
A Dreher1, J Aigner, S Fuchshuber
1Department of Otorhinolaryngology, Ludwig-Maximilians-University, Marchioninistrasse 15, 81377 Munich, Germany.
Archives of Otolaryngology--Head & Neck Surgery
|December 15, 2000
Summary
Relapsing polychondritis, an autoimmune disease affecting cartilage, can present with prolonged, diverse symptoms. This case highlights rare cerebral angiitis as a complication, emphasizing diagnostic challenges.
Area of Science:
- Rheumatology
- Immunology
- Neurology
Background:
- Relapsing polychondritis is a rare chronic inflammatory disorder targeting cartilaginous tissues, eyes, and potentially the central nervous system.
- Autoimmune conditions affecting cartilage can manifest with multisystemic involvement, posing diagnostic challenges.
Observation:
- A 75-year-old woman presented with a 20-year history of dyspnea, stridor, polyarthritis, ear dysmorphism, hearing loss, and tremor.
- Cerebral magnetic resonance imaging revealed multiple spotted signal intensities, suggesting neurological involvement.
Findings:
- Immunohistochemical analysis confirmed antibodies to cartilaginous tissue, specifically type II collagen.
- The patient's prolonged disease course of over 20 years preceded diagnosis.
- Cerebral angiitis was detected, a previously unreported manifestation in this context.
Implications:
- This case underscores the importance of considering relapsing polychondritis in patients with chronic, multisystemic inflammatory symptoms.
- The identification of cerebral angiitis broadens the known spectrum of relapsing polychondritis complications.
- Early diagnosis and recognition of rare neurological involvement are crucial for effective management.