Relapsing Polychondritis

What is relapsing polychondritis?

Relapsing polychondritis is a rare degenerative disease characterized by recurrent inflammation and deterioration of the body’s cartilage. Cartilage is a tough but flexible tissue that covers the ends of bones at a joint, and gives shape and support to other parts of the body. This deterioration may affect any site of the body where cartilage is present.

What causes relapsing polychondritis?

The exact cause of relapsing polychondritis is not known, although it is thought to be an autoimmune disorder. Autoimmune disorders are caused when the body’s natural defenses against “foreign” or invading organisms begin to attack healthy tissue for unknown reasons. In particular, some researchers believe that relapsing polychondritis may be caused by an immunologic sensitivity to type II collagen, a normal substance found in skin and connective tissue.

What are the symptoms of relapsing polychondritis?

The following are the common symptoms associated with the deterioration of cartilage in relapsing polychondritis:

Ear pain, ear swelling, or ear cartilage tenderness
Loss of firmness of the pinna, resulting in a “floppiness”
Hearing impairment
Inflammation of the inner ear, which may also cause:
- Nausea
- Vomiting
- Dizziness
- Ataxia
Joint pain, with or without arthritis
Episcleritis, uveitis and/or scleritis.
- Scleritis may lead to a bluish or dark discoloration of the sclera (white of the eye) and may even be associated with vision loss in severe cases
Proptosis (bulging out of one or both eye balls)
Nasal cartilage inflammation, which may lead to:
- Stuffiness
- Crusting
- Rhinorrhea
- Nose bleeds
- Compromised sense of smell and/or saddle nose deformity
Inflammation of the airways, which may affect the larynx, trachea (windpipe), and bronchi, which in turn may lead to the following:
- Coughing, wheezing, hoarseness, and recurrent infections

Less commonly, relapsing polychondritis may affect the heart, kidneys, nervous system, gastrointestinal tract, and/or vascular (veins) system, causing all kinds of complications.

How is relapsing polychondritis diagnosed?

There are no tests available that are specific for diagnosing relapsing polychondritis. A diagnosis is, therefore, generally based on the presence of characteristic signs and symptoms. Usually, a diagnosis may be reached if the individual has three or more of the following features of the disorder:

Inflammation of the cartilage of both ears
Seronegative polyarthritis (arthritis that involves 5 or more joints simultaneously)
Inflammation of the cartilage of the nose
Eye inflammation (conjunctivitis, episcleritis, scleritis, and/or uveitis)
Inflammation of the cartilage of the airway
Vestibular dysfunction (i.e. vertigo, hearing loss, tinnitus)

A biopsy of affected tissue may be necessary to support the diagnosis.

What are the treatments for relapsing polychondritis?

The primary goals of treatment for people with relapsing polychondritis are to relieve symptoms and to preserve the structure of the affected cartilage. Thus, the main treatment for relapsing polychondritis is corticosteroid therapy with prednisone specifically to decrease the severity, frequency, and duration of relapses. Higher doses are generally given during flares, while lower doses can typically be prescribed during periods of remission. Other medications reported to control symptoms include: