Relapsing polychondritis is characterized by episodes of painful, destructive inflammation of the cartilage and other connective tissues in many organs.

• The ears or nose may become inflamed and tender.
• Other cartilage in the body can be damaged, leading to various symptoms, such as red or painful eyes, hoarseness, cough, difficulty breathing, rashes, and pain in the breastbone.
• Blood tests are done and a piece of tissue may be removed for examination and testing (biopsy).
• If symptoms or complications are moderate or severe, corticosteroids usually help.

This disorder affects men and women equally, usually in middle age. The cause is unknown, but autoimmune reactions to cartilage are suspected.

Symptoms

Typically, one or both ears become red, swollen, and very painful. At the same time or later, a person can develop joint inflammation (arthritis), which may be mild or severe. Cartilage in any joint may be affected, and the cartilage that connects the ribs to the breastbone may become inflamed. Cartilage in the nose is also a common site of inflammation. The nose may become tender, and cartilage can collapse.

Other affected sites include the eyes, resulting in scleritis (inflammation of the white part of the eye), and the voice box (larynx) and windpipe (trachea), resulting in hoarseness, a nonproductive cough, shortness of breath, and tenderness over the Adam's apple. Rarely the cornea may develop a hole (perforate), resulting in blindness. Less often, the heart is involved, leading to heart murmurs and occasionally to heart failure. The skin may become inflamed, resulting in a variety of rashes.

Flare-ups of inflammation and pain last a few weeks, subside, then recur over a period of several years. Eventually, the supporting cartilage can be damaged, resulting in floppy ears; a sloping saddle nose; and vision, hearing, and balance problems.

People who have this disorder may die if the cartilage in their airways collapses, blocking the flow of air, or if their heart and blood vessels are severely damaged.

Diagnosis and Treatment

Relapsing polychondritis is diagnosed when a doctor observes at least three of the following symptoms developing over time:

• Inflammation of both ears
• Painful swelling in several joints
• Inflammation of the cartilage in the nose
• Inflammation of the eye
• Cartilage damage in the respiratory tract
• Hearing or balance problems

A biopsy of the affected cartilage may show characteristic abnormalities. Blood tests, such as the erythrocyte sedimentation rate (ESR), can detect evidence of chronic inflammation.

Mild relapsing polychondritis can be treated with nonsteroidal anti-inflammatory drugs (NSAIDs—see Pain: Nonsteroidal Anti-Inflammatory Drugs) or dapsone. In more severe cases, daily doses of prednisone are given, then tapered off as the symptoms begin to lessen. Sometimes very severe cases are treated with immunosuppressive drugs such as cyclosporine, cyclophosphamide, or azathioprine. These drugs treat the symptoms but have not been shown to alter the ultimate course of the disorder.

Last full review/revision February 2008 by Rula A. Hajj-Ali, MD