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Relapsing Polychondritis

by Rula A. Hajj-ali, MD

Relapsing polychondritis is a rare disorder 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 around the breastbone.

  • Blood and laboratory tests, imaging, and removal of a piece of tissue for examination and testing may be done.

  • If symptoms or complications are moderate or severe, corticosteroids and immunosuppressants 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 ear or both ears (but not the ear lobes) 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 inflammation. Rarely, the cornea may develop a hole (perforation), resulting in blindness. The voice box (larynx), windpipe (trachea), or airways of the lungs can be affected, resulting in hoarseness, a nonproductive cough, shortness of breath, and tenderness over the Adam’s apple. Less often, the heart is involved, leading to heart murmurs and occasionally to heart failure. Rarely, the kidneys are affected.

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 a hollow at the lower part of the chest (pectus excavatum). The nerve in the inner ear can be affected, causing eventual problems with balance and hearing, and eventually vision problems can occur.

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

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

  • Inflammation of both outer 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 (most often from the ear) may show characteristic abnormalities. Blood tests, such as the erythrocyte sedimentation rate, can detect evidence of inflammation. Blood tests also reveal whether the person has a low number of red blood cells or a high number of white blood cells and whether certain antibodies are present.

Doctors also evaluate the airways with spirometry (see Pulmonary Function Testing (PFT)) and computed tomography (CT) of the chest.

Prognosis and Treatment

About 94% of people survive at least 8 years after the diagnosis is made. Newer therapies have decreased the rate of death.

Mild relapsing polychondritis of the ear can be treated with nonsteroidal anti-inflammatory drugs (NSAIDs—see see Nonopioid Analgesics : Nonsteroidal Anti-Inflammatory Drugs) or dapsone. However, most people are given a dose of the corticosteroid prednisone that is gradually decreased as the symptoms begin to lessen. In some people, symptoms do not lessen, so the dose of corticosteroids cannot easily be decreased. These people may also be given methotrexate to reduce the need for corticosteroids. Sometimes very severe cases are treated with immunosuppressive drugs such as cyclosporine, cyclophosphamide, or azathioprine or drugs that inhibit a chemical called tumor necrosis factor (for example, infliximab or etanercept). These drugs treat the symptoms but have not been shown to alter the ultimate course of the disorder.

Surgery may be needed to correct collapse or narrowing of the trachea.

People who take corticosteroids are at risk of fractures related to osteoporosis. To prevent osteoporosis, these people are given the drugs used to treat osteoporosis, such as bisphosphonates and supplemental vitamin D and calcium. People who have relapsing polychondritis are at increased risk of atherosclerosis (see Atherosclerosis) and are closely monitored by doctors. People who are receiving immunosuppressants are also given drugs to prevent infections such as by Pneumocystis jirovecii.

Drugs Mentioned In This Article

  • Generic Name
    Select Brand Names
  • ENBREL
  • ACZONE
  • REMICADE
  • CYTOXAN (LYOPHILIZED)
  • OTREXUP
  • RAYOS
  • NEORAL, SANDIMMUNE
  • IMURAN