Relapsing polychondritis is a rare disease. It causes recurrent swelling or inflammation of the cartilage and other tissue throughout a person’s body. Scientists are unsure of the cause of relapsing polychondritis but consider it an autoimmune disorder.

Relapsing polychondritis is a rare condition that affects less than 5,000 people in the United States.

It causes inflammation or swelling of the cartilage in a person’s body. Cartilage is a tough but flexible tissue in areas such as the ears and nose. The disease can affect any area containing cartilage.

Relapsing polychondritis can also affect other tissue, organs, and systems, including the eyes, heart, and respiratory system.

This article reviews relapsing polychondritis, including its symptoms, causes, risk factors, and more.

A person holding their legs which are inflamed. Relapsing polychondritis causes inflammation of the body's cartilage and joints.Share on Pinterest
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Relapsing polychondritis can cause different symptoms based on the areas it affects.

The first symptoms a person often experiences are sudden pain, tenderness, and swelling of the cartilage of the outer ear. Recurrent attacks can also affect the middle ear and lead to hearing loss.

Other common symptoms include:

Symptoms may also come and go in what is known as flares. A flare’s severity and occurrence will vary from person to person. They may last for a few days to a few weeks before subsiding.

If enough cartilage in the nose is affected, it can cause the bridge of the nose to collapse into a saddle nose shape.

Other systems relapsing polychondritis may affect include:

  • cardiovascular system, including the lungs and heart
  • kidneys
  • blood vessels

Learn more about the early symptoms of relapsing polychondritis.

The exact underlying cause of relapsing polychondritis is not known.

Experts currently consider it to be an autoimmune disorder. This means that a person’s immune system mistakenly attacks healthy cells, causing inflammation. Some symptoms may occur when the immune system attacks the cartilage.

Some experts believe that an immune response to type II collagen — found in connective tissues throughout the body — may be the underlying cause.

Similar to causes, the risk factors of relapsing polychondritis are not well understood or known. Some suspect that genetic makeup and environmental triggers may cause relapsing polychondritis symptoms.

The condition affects men and women equally, and symptoms usually begin between 40 and 60 years.

According to a small 2022 study, researchers found that possible risk factors for the recurrence of symptoms include:

  • tracheal involvement — the airway that leads from a person’s voicebox to their windpipe
  • initial treatment with prednisolone only — a corticosteroid medication
  • pretreatment C-reactive protein level

They suggested that early treatment with a combination of prednisolone and immunosuppressants may help prevent recurrences.

Diagnosing relapsing polychondritis can be challenging, as it is a very rare disease that is not well known by the medical community.

Inflammation comes and goes, and the symptoms it tends to cause are nonspecific, meaning it can appear similar to other conditions.

For example, ear pain is often the first symptom of the disease. A healthcare professional may assume it is an ear infection and treat it with antibiotics. Since the symptoms may clear on their own, they may presume that the antibiotics worked and not investigate further.

It may take several episodes of inflammation and symptom presentation before healthcare professionals determine relapsing polychondritis.

There is no specific test to diagnose relapsing polychondritis. However, healthcare professionals may order tests to rule out other conditions. They will also gather information from the person about their symptoms, including:

  • when they occur
  • severity
  • duration
  • where they occur

Once a person receives a diagnosis of relapsing polychondritis, a healthcare professional will likely recommend the following medications:

The small 2022 study noted above found that a combination of immune-suppressive medications and steroids helped prevent recurrence in participants.

In extreme cases affecting the heart, a healthcare professional may recommend replacing heart valves. If relapsing polychondritis affects the trachea, a person may require steroid injections into the area to prevent deterioration. However, doctors may recommend the insertion of a breathing tube if the trachea is significantly damaged.

Learn more about treatment options for relapsing polychondritis.

The outlook for people with relapsing polychondritis has improved In recent years. A 2022 article notes that survival rates have risen from 70% at 5 years to 91% at 10 years.

With an accurate diagnosis and treatment, a person should be able to live a full life.

A person may need to seek second opinions and consult healthcare professionals prepared to talk about their symptoms to help get an accurate diagnosis as soon as possible.

Early diagnosis and treatment can help improve outcomes for people with relapsing polychondritis. If the condition does not receive prompt treatment, it may cause irreversible tissue damage and result in complications.

Symptoms of relapsing polychondritis may appear similar to the following conditions:

Relapsing polychondritis is a rare condition that experts consider to be an autoimmune disorder. It often causes cartilage inflammation but may also affect other organs and systems.

Symptoms are generally nonspecific and sporadic. Combined with being a rare condition, it can make diagnosis challenging.

Treatments often involve using steroids or immunosuppression to help improve symptoms and prevent them from reoccurring. With treatment, a person can generally live a healthy life.