Section 5. Bone, Joint, and Muscle Disorders

Chapter 68. Autoimmune Disorders of Connective Tissue

Topics: Introduction | Systemic Lupus Erythematosus | Scleroderma | Sjögren's Syndrome | Polymyositis and Dermatomyositis | Mixed Connective Tissue Disease | Relapsing Polychondritis | Eosinophilic Fasciitis

Relapsing Polychondritis

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

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 conjunctivitis; rarely the cornea may become perforated, resulting in blindness; voice box (larynx) and windpipe (trachea), resulting in hoarseness, a nonproductive cough, and tenderness over the Adam's apple; and bronchi, sometimes resulting in pneumonia. Less often, the heart is involved, leading to heart murmurs and occasionally to heart failure. Blood vessels may become inflamed; when blood vessels in the brain are involved, seizures and strokes may occur. The kidneys may become inflamed, sometimes leading to kidney 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, and hearing or balance problems.

A biopsy of the affected cartilage may show characteristic abnormalities. Blood tests, such as the erythrocyte sedimentation rate (ESR), which measures the rate at which red blood cells settle to the bottom of a test tube containing blood, can detect evidence of chronic inflammation.

Mild relapsing polychondritis can be treated with aspirin or other nonsteroidal anti-inflammatory drugs (NSAIDs) (see Section 6, Chapter 78). In more severe cases, daily doses of prednisone are given, then tapered off as the symptoms begin to improve. Sometimes very severe cases are treated with immunosuppressive drugs such as methotrexate or cyclophosphamide. These drugs treat the symptoms but have not been shown to alter the ultimate course of the disorder.