Relapsing Polychondritis

What is relapsing polychondritis?

Relapsing polychondritis is a disorder in which defects develop in cartilage and other tissues throughout the body, including the ears, nose, eyes, joints, and respiratory tract. It is considered a rare condition.

Symptoms vary widely by case, but you may experience ear pain, redness, and structural changes, hearing loss or other ear impairments, eye pain, vision loss, and other eye problems, nose deformities and other nasal and respiratory issues such as trouble breathing, joint pain, chest discomfort, and full-body swelling.

Since this is a chronic condition, treatment focuses on alleviating symptoms and associated disorders.

You should visit your primary care physician for mild severity of relapsing polychondritis. Medications such as NSAIDs and steroids are generally prescribed.

Relapsing polychondritis symptoms

Several different body structures and systems will be affected in cases of relapsing polychondritis. including the ears, eyes, the nose, the respiratory system, joints, and chest.

Ear-related symptoms

A variety of ear-related symptoms and inflammation will likely develop, resulting in:

• Pain: This may develop suddenly or gradually, and may last for days or weeks.
• Redness: The ear may turn a red or purple color, but the ear lobe usually remains normally colored because it does not contain cartilage.
• Structural changes: These changes will likely be long-term. Repeated inflammation can cause changes to the structure of the ear, making it look floppy.
• Hearing loss, ringing in the ears, or vertigo: About one-third of people with relapsing polychondritis will eventually develop hearing loss, ringing in the ears (tinnitus), or vertigo. This can occur if the inflammation spreads to the inside of the ear to the structures responsible for hearing and balance.

Eye-related symptoms

About half of people with relapsing polychondritis will experience eye symptoms over the course of their disease. These symptoms include:

• Eye pain or redness
• Blurry vision
• Vision loss
• Swelling behind the eye

Nasal symptoms

About half of people with relapsing polychondritis will experience nasal symptoms over the course of the disease, including:

• Stuffy nose
• Runny nose
• Bloody nose
• Impaired sense of smell
• Deformities of the nose: Repeated or prolonged inflammation can change the shape of the nose, causing a "saddle nose deformity" in which the top of the nose is caved in.

Relapsing polychondritis causes

The cause of relapsing polychondritis is unknown, but some factors are thought to contribute to the development of relapsing polychondritis. These factors include genetic mutations, other immune system disorders, recreational drug use, and infections.

Genetic mutations

Certain genetic mutations are thought to increase the risk of developing relapsing polychondritis. These include mutations in genes known as "human leukocyte antigens," which are proteins found on certain cells of the immune system.

Other immune system disorders

Relapsing polychondritis is often associated with other disorders of the immune system. It is believed that these immune system disorders may create an imbalance that causes the immune system to attack the body's own cartilage and tissues. These include disorders known as:

• Vasculitis: Inflammation of blood vessels
• Graves' disease: An autoimmune disease of the thyroid gland
• Ulcerative colitis: An autoimmune disease of the gastrointestinal tract

Recreational drug use

Cases of relapsing polychondritis have been reported to occur after episodes of recreational drug use. The drug or other chemicals mixed into the drugs may either directly damage the cartilage tissue, or create an imbalance in the immune system that causes damage to the cartilage tissue.

Infections

Some infections may contribute to the development of relapsing polychondritis by either directly damaging cartilage tissue, or by triggering the immune system to attack the body's own cartilage.

Treatment options and prevention

Relapsing polychondritis is a chronic disorder that cannot be completely cured. Therefore, treatment is focused on reducing symptoms and treating associated disorders. Specific treatment options include anti-inflammatory medications, steroid injections for joint pain, vaccinations, surgery to treat airway or heart problems, and surgery to repair deformities in your appearance.

Anti-inflammatory medications

This is the primary form of medical treatment for relapsing polychondritis. The specific medication your physician may recommend will depend on the severity of symptoms and your response to other medications.

• NSAIDs: People with mild or moderate disease without involvement of internal organs will usually be treated with nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen (Advil) or naproxen (Aleve).
• Oral or topical steroids: People who do not respond to NSAIDs may be started on steroids such as prednisone or another anti-inflammatory medication called dapsone (Aczone).
• Other anti-inflammatories: People who do not respond to either NSAIDs or steroids may be started on other anti-inflammatory medications including methotrexate, azathioprine (Imuran), cyclophosphamide or cyclosporine, among others.

Steroid injections for joint pain

People who are experiencing joint pain may benefit from steroid injections to the inflamed joints. These may be repeated at regular intervals until the inflammation subsides.

When to seek further consultation

You should seek medical attention if you have reason to believe you have relapsing polychondritis or you have trouble breathing.

If you develop any symptoms of relapsing polychondritis

You should go see your physician if you experience inflammation of the ears, nose, or eyes, trouble breathing, or joint pain.

If you are having trouble breathing

You should consider going directly to the emergency room if you are experiencing trouble breathing. Your physician can do an examination and order testing to determine if you have relapsing polychondritis as well as any disorders that are classically associated with relapsing polychondritis, and subsequently offer the appropriate treatment.

Questions your doctor may ask to diagnose

• Any fever today or during the last week?
• Have you been feeling more tired than usual, lethargic or fatigued despite sleeping a normal amount?
• Do you have a cough?
• Have you lost your appetite recently?
• Have you experienced any nausea?

Self-diagnose with our free Buoy Assistant if you answer yes on any of these questions.