Polychondritis


Polychondritis

Description:

Inflammation of cartilage.

Types: Chronic atrophic polychondritis and relapsing polychondritis.

ICD-9: 733.98.

Polychondritis is a chronic, recurrent, episodic, inflammatory disease of unknown cause affecting predominantly the cartilaginous tissues of the body. Episodes last few days to weeks. All types of cartilage: elastic cartilage (ears and nose), hyaline cartilage (peripheral joints), fibrocartilage (at axial sites) and cartilage in the tracheobronchial tree and proteoglycan rich structures (eye, heart, blood vessels, and inner ear) may be involved).

Symptoms:

Common symptoms include:

    Fatigue or malaise.

  • Red, swollen, painful ears, hearing loss, dizziness.

  • Fever.

  • Ears that are floppy, that is, they are softer than normal, limp, or droppy.

  • Inflammation over the bridge of the nose, nasal congestion.

  • Arthritis.

  • Shortness of breath, cough, stridor (high-pitched sound during breathing).

  • More rarely: Eye inflammation, leaky heart valves, kidney disease, neurological problems, rash.

Causes and Risk Factors:

The cause of relapsing polychondritis is unknown. It is suspected that this condition is caused by an immune system disorder in which the body's immunity system (which normally fights off invaders of the body, particularly infections) is misguided. This results in inflammation that is directed at various tissues of the body.

Polychondritis is a rare disease in which cartilage in many areas of the body becomes inflamed. The disease most commonly affects the ears, nose and the airways of the lungs. The cause is not known, and it occurs most often in people in their 50s or 60s. One theory is that polychondritis might be an autoimmune disease, in which the immune system attacks the body rather than foreign invaders such as viruses. Relapsing polychondritis affects men and women equally; onset typically is in middle age.

In polychondritis, it's possible that a triggering event, perhaps an infection, sets off a reaction by the immune system, which unleashes an attack on the body's cartilage. Some people may have a genetic makeup that makes them more prone to this. The disease does not seem to run in families. It sometimes appears in people who have other disease, such as rheumatoid arthritis, vasculitis (inflammation of blood vessels) and systemic lupus erythematosus (SLE or lupus).

Diagnosis:

There is no one specific test for diagnosing relapsing polychondritis. Blood tests that indicate inflammation, such as an elevated erythrocyte sedimentation rate (ESR), C-reactive protein, and others, are often abnormal when the disease is active.

A biopsy of an inflamed area is required to confirm the diagnosis. In a biopsy, a small piece of tissue is removed and examined under a microscope. The biopsy also is helpful to rule out other causes of symptoms such as infection, especially tuberculosis, syphilis, leprosy and fungal disease. Sometimes, the biopsy is not conclusive, or the doctor is certain enough of the diagnosis that a biopsy does not have to be done. No single blood test will give a definite diagnosis, including tests for antibodies to cartilage. Imaging studies, including a computed tomography (CT) scan of the lungs and other tests, especially pulmonary-function tests, may be done to help determine the extent of disease.

However, if a biopsy is not obtained, the diagnosis is still likely when a person has at least three of the following:

    Inflammation of the cartilage in both ears.

  1. Arthritis of multiple joints.

  2. Inflammation of the bridge of the nose.

  3. Eye inflammation.

  4. Inflammation of the cartilage in the trachea or throat.

  5. Neurosensory hearing loss, tinnitus, and vertigo.

Treatment:

Polychondritis is a chronic (long-lasting) disease, although medications frequently can reduce the severity of symptoms. Sometimes, the disease goes into remission, meaning it goes away temporarily, whether or not the person is treated.

For patients with more mild disease, nonsteroidal antiinflammatory drugs (NSAIDs), including ibuprofen (Motrin), naproxen (Naprosyn), and others, can be helpful to control the inflammation. Usually, however, cortisone-related medications (steroids such as prednisone and prednisolone) are required. High-dose steroids are frequently necessary initially, especially when the eyes or breathing airways are involved. Moreover, most patients require steroids for long-term use.

Methotrexate (Rheumatrex, Trexall) has shown promise as a treatment for relapsing polychondritis in combination with steroids as well as a maintenance treatment. Studies have demonstrated that methotrexate can help reduce the steroid requirements.

Other medications that have been tried in small numbers of patients with some reports of success include cyclophosphamide (Cytoxan), dapsone, azathioprine (Imuran), penicillamine (Depen, Cuprimine), cyclosporine, and combinations of these drugs with steroids.

Note: The following drugs and medications are in some way related to, or used in the treatment. This service should be used as a supplement to, and NOT a substitute for, the expertise, skill, knowledge and judgment of healthcare practitioners.

Because the disease is so rare, studies to determine the single best treatment are difficult to do. As a result, there is no clear ideal therapy for this disease. Treatment depends on what parts of the body are affected and how severely.

Tracheal surgery or stenting and treatment of any accompanying infection also are options to be considered in some cases. Surgery to repair an injured part of the trachea is a major operation. Increasingly, however, doctors use a procedure called bronchoscopy, in which they use a flexible tube to look down the throat and into the trachea, and insert a small plastic tube called a stent to keep the airway open. When the person also has an infection, such as pneumonia or bronchitis, antibiotic therapy can be lifesaving.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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