Relapsing Polychondritis

What is relapsing polychondritis?

Relapsing polychondritis is a rare disorder that causes inflammation and deterioration of cartilage. Cartilage is a connective tissue throughout your body.

Relapsing polychondritis tends to affect the cartilage in your:

• Ears.
• Nose.
• Larynx (voice box).
• Trachea (windpipe).

Other names for relapsing polychondritis include:

• Chronic atrophic polychondritis.
• Generalized or systemic chondromalacia.
• Meyenburg-Altherr-Uehlinger syndrome.
• Relapsing perichondritis.
• Von Meyenburg disease.

Who might get relapsing polychondritis?

Relapsing polychondritis can affect anyone, but it usually starts in people ages 40 to 60.

How common is relapsing polychondritis?

This condition is rare. Researchers estimate that it affects 1 in 285,000 people.

What causes relapsing polychondritis?

Scientists don’t know what causes relapsing polychondritis. Some consider it an autoimmune disease (when your immune system attacks healthy tissue). But they don’t understand why it happens.

What are the symptoms of relapsing polychondritis?

Relapsing polychondritis tends to occur in episodes or flares. An episode may last a few days or weeks, and they occur repeatedly for years.

The first symptoms are usually pain, tenderness, swelling and redness in one or both ears. The symptoms affect cartilage inside and outside your ears but not your ear lobes. Your ears may become floppy.

Other relapsing polychondritis symptoms include:

• Cartilage collapse at the bridge of your nose, which may result in saddle nose deformity.
• Joint pain similar to arthritis.
• Nasal congestion.
• Pain near your breastbone.
• Pain and redness in your eyes.
• Hearing loss, tinnitus or vertigo if your inner ear is affected.
• Difficulty breathing if it affects your trachea or trouble speaking if it affects your larynx.

Rarely, someone with relapsing polychondritis might also have:

• Heart valve.
• Heart murmur.
• Vision loss.
• Kidney disease.

How is relapsing polychondritis diagnosed?

There’s no test to diagnose or confirm relapsing polychondritis. A healthcare provider can diagnose the condition if you have at least three associated symptoms over time. A biopsy of your ear cartilage or a blood test might help confirm a diagnosis.

How is relapsing polychondritis treated?

There’s no cure for this condition. Relapsing polychondritis treatment aims to ease symptoms and reduce the chances of complications. Treatment often begins with:

• Colchicine (often used for gout).
• Corticosteroids such as prednisone.
• Dapsone (an anti-infective).
• Nonsteroidal anti-inflammatory drugs (NSAIDs).

In more severe cases, healthcare providers may prescribe:

• Medications that suppress the immune system, such as cyclophosphamide or azathioprine.
• Methotrexate (a type of chemotherapy used against arthritis).

People with very severe cases may require surgery, such as:

• Heart valve surgery.
• Insertion of a breathing tube (tracheostomy).
• Procedures to open the airway. This may include inserting a laryngeal or tracheal stent, which props open the airway, or airway dilation, which uses a balloon to stretch the airway.
• Surgery to reconstruct the trachea or larynx.

How can I prevent relapsing polychondritis?

Scientists don’t understand what causes relapsing polychondritis, so there are no strategies to prevent it.

Is relapsing polychondritis fatal?

Relapsing polychondritis life expectancy is generally good. Almost 95% of people with the condition are alive eight years after diagnosis.

But it can cause life-threatening complications, particularly breathing problems. And it can cause permanent loss of hearing or vision.

How long will I have relapsing polychondritis?

Relapsing polychondritis is a chronic condition that doesn’t go away. But with treatment, you may be able to make episodes less intense and less frequent.

What else can I ask my healthcare provider?

If you’ve been diagnosed with relapsing polychondritis, consider asking your healthcare provider the following questions:

• How severe is my condition?
• What medications do you recommend?
• Should I have tests to check my vision, hearing, airway, heart or kidneys?
• Should I see any specialists?
• Can I do anything to strengthen my cartilage or slow down the degeneration?
• Do you know of any clinical trials for this condition?

When should I seek medical attention?

If you have relapsing polychondritis, schedule regular appointments with your healthcare providers. If you have any issues with breathing, vision or hearing, seek medical attention as soon as possible. Don’t wait for your next appointment.

A note from Cleveland Clinic

Relapsing polychondritis is a rare disorder that causes inflammation and deterioration of cartilage. It usually affects your ears and nose, but it can damage your airway and other body parts. If you have symptoms of this condition, talk to a healthcare provider. Most cases are manageable, but life-threatening complications can occur.