What Is Relapsing Polychondritis?

If you have painful joints and your ears or nose are red or don’t look quite right, you might have relapsing polychondritis (RP), a rare disease that causes inflammation.

Inflammation is your body’s way to fight disease or injury. When your immune system thinks there’s a problem (like a virus or bacteria), it releases certain cells in your blood, and more blood flows to the affected area. That can cause redness, warmth, swelling, or pain.

The pain from RP usually comes on suddenly, and it can happen to both men and women, and to people of all ages. But it’s more likely to start between the ages of 40 and 60.

It affects people in different ways. Some get a mild case of RP once in a while, and the symptoms go away on their own. Others have lots of pain and more frequent attacks.

Because it can affect key organs, RP can lead to serious illness and can be life-threatening.

Cause

Doctors don’t know what causes RP. Some think a certain gene may make you more likely to get it, but it doesn’t run in families.

It’s considered an autoimmune disorder. That means your immune system attacks healthy tissue by mistake. Researchers think some cases might be triggered by stress or things in the environment.

Symptoms

The disease mostly affects cartilage (firm but flexible tissue) in your ears and joints. It also may show up in your nose, ribs, spine, and windpipe. It can affect any area where the tissue is similar to cartilage, like your eyes, heart, skin, kidney, ribs, blood vessels, and nervous system.

Common signs of relapsing polychondritis include:

  • A dip in the bridge of your nose ("saddle nose" or "pug nose")
  • Ear pain and redness
  • Red, painful, and swollen eyes
  • Painful, swollen joints (hands, fingers, shoulders, elbows, knees, ankles, toes, pelvis)
  • Rib pain
  • Throat or neck pain
  • Trouble breathing and speaking
  • Trouble swallowing
  • Rashes

Depending on where RP affects you, it can cause problems with a heart valve or kidney issues as well. If RP affects your inner ear, you could feel sick to your stomach or have trouble with hearing and balance.

If the disease gets into your windpipe, it can cause a cough and make it hard to breathe or swallow. You also can have severe RP pain in your breastbone and ribs.

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Diagnosis

There isn’t a test for RP. Your doctor will examine you and ask questions about your symptoms. He might ask you to get a blood test to look for signs of inflammation or X-rays so he can see the affected areas better.

To find out for sure if you have RP, your doctor might look for three or more of these:

  • Inflammation of the cartilage in both your ears
  • Inflammation of the cartilage in your nose
  • Inflammation of the cartilage in your airway
  • Arthritis in five or more joints at the same time
  • Hearing or balance problems.

In some cases, your doctor may want to take a small amount of tissue to look at under a microscope. This is known as a biopsy.

Depending on how the disease affects you, you might need to see a specialist. This could be an expert in autoimmune disorders (rheumatologist), heart problems (cardiologist), or pain management.

Treatment

There’s no cure for RP, but your doctor can help you feel better and save your cartilage. Anti-inflammatories (like Motrin or Advil) can help with pain, especially for people who have a mild case of RP.

Your doctor also might suggest steroids (like prednisone) or other kinds of drugs to help with inflammation.

In severe cases, she might recommend stronger drugs that slow down your immune system. And depending on which organs are affected, you might need surgery to fix a damaged heart valve or put in a breathing tube.

WebMD Medical Reference Reviewed by Lisa Bernstein, MD on December 26, 2016

Sources

SOURCES:

National Institutes of Health, National Center for Advancing Translational Sciences, Genetic and Rare Diseases Information Center: “Relapsing polychondritis.”

National Organization for Rare Disorders: “Relapsing Polychondritis.”

Relapsing Polychondritis Awareness and Support Foundation: “Collaboration Announcement,” “Frequently Asked Questions,” “Relapsing Polychondritis,” “What Happens to Patients with Relapsing Polychondritis?”

Up to Date: “Clinical manifestations of relapsing polychondritis,” “Treatment of relapsing polychondritis.”

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