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Relapsing Polychondritis

By

Alana M. Nevares

, MD, The University of Vermont Medical Center

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Relapsing polychondritis is a rare autoimmune rheumatic 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, and established criteria may be used for diagnosis.

  • 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 of relapsing polychondritis 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.

Relapsing Polychondritis (Ear)
Relapsing Polychondritis (Ear)
© Springer Science+Business Media

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

  • Established criteria

  • Sometimes biopsy

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 but is not required for diagnosis.

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. Although blood test results can help doctors diagnose relapsing polychondritis, they alone cannot confirm a definite diagnosis of relapsing polychondritis because sometimes the abnormalities they detect are present in healthy people or in people who have other disorders. The diagnosis of relapsing polychondritis is based on all of the information doctors gather, including symptoms, physical examination results, and all test results.

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

Prognosis

People with relapsing polychondritis tend to die earlier than they otherwise would, most often because of damage to the heart, lungs, or blood vessels. Newer therapies have decreased the rate of death.

Treatment

  • NSAIDs or dapsone for relapsing polychondritis of the ear

  • Corticosteroids

  • Sometimes immunosuppressive drugs

Mild relapsing polychondritis of the ear can be treated with nonsteroidal anti-inflammatory drugs (NSAIDs) 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 are receiving immunosuppressants are also given drugs to prevent infections such as by the fungus Pneumocystis jirovecii (see prevention of pneumonia in immunocompromised people).

People who have relapsing polychondritis are at increased risk of atherosclerosis and are closely monitored by doctors.

Drugs Mentioned In This Article

Generic Name Select Brand Names
CYTOXAN (LYOPHILIZED)
IMURAN
NEORAL, SANDIMMUNE
OTREXUP
REMICADE
RAYOS
ENBREL
ACZONE
Last full review/revision April 2018 by Alana M. Nevares, MD
NOTE: This is the Consumer Version. DOCTORS: Click here for the Professional Version
Click here for the Professional Version
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