Relapsing polychondritis is a rare disease that causes inflammation of your cartilage. It most commonly affects your ears, joints, and nose.
Researchers don’t clearly understand why some people develop the rare inflammatory disease known as relapsing polychondritis, but they believe it occurs when your immune cells attack healthy cartilage cells.
People with relapsing polychondritis have flare-ups of pain or swelling in the affected areas followed by periods of remission where symptoms subside. The outlook for people with relapsing polychondritis tends to be good. But some people develop respiratory or cardiovascular problems that can be life threatening.
This article will explain this rare condition and provide more detail on symptoms, why this happens, and how you can treat this disease.
“Relapsing” means the condition causes reoccurring flare-ups of symptoms. “Polychondritis” means that it causes problems with many different areas of cartilage.
The World Health Organization (WHO) classifies this as a chondropathy, which is a disease of your cartilage. Cartilage is a hard connective tissue that has many functions in your body such as:
- reducing friction between your joints
- absorbing shock
- giving structure to your nose and ears
Over time, relapsing polychondritis can cause
Relapsing polychondritis usually begins with sudden pain or tenderness in the cartilage in one or both ears. Flare-ups may last days to weeks.
Ear symptoms develop in about
You may develop symptoms such as:
Some people develop a collapse of their ear canal or inflammation of the structures inside the ear. Damage to these structures can cause hearing loss and associated symptoms:
More than 70% of people develop painful and swollen joints lasting from weeks to months. Symptoms tend to be similar to those of arthritis. About 30% of people experience joint pain as the first symptom.
More than 50% of people with relapsing polychondritis develop nose symptoms at some point. It can lead to sudden pain and tenderness around the soft part of your nose.
In some cases, the nose can become severely damaged and collapse. This condition is known as saddle nose.
- eyeball protrusion
- conjunctivitis (pink eye)
- corneal ulcers
- red eyes
- inflammation of the middle layer of your eye (uveitis)
- swelling around your eyes
Cardiovascular problems develop in 25% to 50% of people with relapsing polychondritis and can be serious. Symptoms might include
- inflammation of large blood vessels
- valve disease in your heart
- airway collapse
- nasal or sinus problems
- trouble breathing
Respiratory complications are the
Central nervous system symptoms
Involvement of the brain and spinal cord occurs in about
- brain inflammation and swelling
- cognitive dysfunction
- memory loss
- hearing loss
Relapsing polychondritis can be difficult to diagnose since no single blood or imaging test can provide a definitive diagnosis. It can also mirror symptoms from other conditions affecting different parts of the body.
The average time to receive a diagnosis from symptom onset has been reported as
Doctors usually diagnose relapsing polychondritis based on your signs and symptoms.
- Reoccurring inflammation in the cartilage of both ears
- Inflammation of your nose cartilage
- Nonerosive inflammatory arthritis, meaning there’s no loss of the cartilage at the end of the bone or the bone underneath
- Inflammation of the middle or outer layers of your eye
- Inflammation of the cartilage in your respiratory tract including the cartilage of your voice box or windpipe
- Damage to the structures of your inner ear that causes at least one of:
- hearing loss
A doctor will likely want to perform a computed tomography (CT) scan to look for airway changes if they suspect you have relapsing polychondritis. They might also order:
- positron emission tomography (PET)-CT imaging
- biopsy of an affected joint
- blood tests
It’s not known what causes relapsing polychondritis, but it’s thought to be an autoimmune disease. People sometimes have antibodies against
Like with many other autoimmune conditions, researchers think that it may occur when people who are genetically predisposed are exposed to triggering factors such as:
direct traumato the ear
No standard therapy has been developed to treat relapsing polychondritis. Treatment largely revolves around treating symptoms.
Mild disease might be manageable with medications to reduce inflammation such as:
- nonsteroidal anti-inflammatory drugs (NSAIDs)
In severe cases, corticosteroids such as oral prednisolone or rarely methylprednisolone administered through an IV may be needed to decrease inflammation.
Biologic drugs may be recommended for people who can’t take corticosteroids or when corticosteroids
Researchers aren’t entirely sure why relapsing polychondritis develops, and there’s no cure. It’s likely that a combination of genetics and environmental factors plays a role.
You can potentially reduce your number of flare-ups by making regular follow-ups with a doctor and taking your medication as prescribed.
You might also find it helpful to keep a diary of when your flare-ups occur along with potential triggers you come into contact with. Over time, you may notice patterns of when you have flare-ups occur and develop a better understanding of what might be triggering them.
The outlook for people with relapsing polychondritis is generally good. About 91% of people survive at least
Relapsing polychondritis is a rare condition that causes the breakdown of cartilage in parts of your body such as your ears, nose, and joints. Most people have a good outlook, but some people can develop life threatening complications such as the collapse of their windpipe.
If a doctor suspects you have relapsing polychondritis, they can order tests to help rule out other conditions. They can also help you develop a treatment plan to reduce your number of flare-ups.