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Relapsing polychondritis in an adolescent
R J Trepel1, R N Lipnick, L D'Angelo
1Division of Adolescent and Young Adult Medicine, Children's Hospital National Medical Center, Washington, D.C.
Summary
Relapsing polychondritis (RP) is a rare systemic disease affecting cartilage. This case highlights its variable presentation and the critical need for prompt diagnosis and aggressive treatment in adolescents to prevent severe complications.
Area of Science:
- Rheumatology
- Internal Medicine
- Systemic Autoimmune Diseases
Background:
- Relapsing polychondritis (RP) is a rare systemic autoimmune disorder characterized by episodic inflammation of cartilaginous tissues.
- It affects various organs, including the ears, nose, joints, and respiratory tract, with a highly variable clinical course.
Observation:
- A 17-year-old female presented with depression, weight loss, chest pain, hoarseness, and dysphagia, progressing to respiratory distress.
- Physical findings included cachexia, fever, pharyngeal erythema, and tenderness in the auricle, chest wall, cricothyroid cartilage, and knees.
- Radiological and biopsy findings confirmed RP, with tracheal cartilage calcification, subglottic stricture, and saddle nose deformity.
Findings:
- The patient experienced auricular chondritis, inflammatory arthritis, nasal chondritis, respiratory tract chondritis, and audiovestibular abnormalities.
- Despite initial treatment with corticosteroids, dapsone, and cyclophosphamide, respiratory distress necessitated a tracheostomy.
- RP can present in adolescents, with a significant percentage of cases involving chondritis and arthritis.
Implications:
- Early and aggressive management of relapsing polychondritis is crucial for reducing morbidity and mortality.
- This case underscores the importance of recognizing RP in younger populations and considering its diverse manifestations.
- Prompt diagnosis and multimodal therapy are essential for managing this rare and potentially life-threatening condition.