Relapsing Polychondritis

September 14, 2005
Charles E. Crutchfield III, MD

,
Eric J. Lewis, MD

Relapsing polychondritis, as manifested in one of this 55-year-old man's deformed ears, is a rare, chronic, autoimmune inflammatory disease of cartilaginous structures. The disease also affected the cartilage in this patient's nose, which is the second most common site of involvement. Tissues of the joints, eyes, and blood vessels as well as the trachea and the bronchial tree may also be affected and destroyed.

Relapsing polychondritis, as manifested in one of this 55-year-old man's deformed ears, is a rare, chronic, autoimmune inflammatory disease of cartilaginous structures. The disease also affected the cartilage in this patient's nose, which is the second most common site of involvement. Tissues of the joints, eyes, and blood vessels as well as the trachea and the bronchial tree may also be affected and destroyed.

Chondritis of the external ears, nose, or respiratory tract; inflammatory polyarthritis; ocular inflammation; or auditory or vestibular dysfunction is characteristic of relapsing polychondritis. Tissue from a biopsy of an affected site can confirm the diagnosis.

The course of relapsing polychondritis is unpredictable, but approximately 30% of patients die within 4 years of the diagnosis, usually from respiratory tree complications or vascular aneurysms. Some patients experience frequent exacerbations and remissions; others may suffer a few episodes and recover; while still others have episodes that lead to severe deformities.

Current therapies are suboptimal. Topical treatments are ineffective; systemic options that may be tried include prednisone, NSAIDs, methotrexate, and dapsone.

This patient had several recurrences of the disease, which left him with ear and nose deformities. The condition eventually cleared without additional morbidity.

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