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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.

Journal of Adolescent Health Care : Official Publication of the Society for Adolescent Medicine

|November 1, 1989

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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.

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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.