Diseases

Relapsing Polychondritis

Relapsing polycondrosis is very rare autoimmune disorder that causes inflammation in the cartilaginous tissues throughout the body. Cartilage is a type of connective tissue that is tough and flexible.

The most common cartilaginous tissues affected by relapsing polychondritis are in the ears, nose, eyes, joints and respiratory tract.

Although relapsing polychondritis is a chronic (long-term) disease, it tends to flare and can get better for long periods between flares.

Most cases are mild and can be well managed with appropriate care. However, if inflammation caused by the disease is not controlled, it can eventually cause permanent damage to the cartilage that cannot be repaired.

Relapsing polychondritis can be associated with other serious autoimmune conditions such as vasculitis and connective tissue disease. When this is the case, associated conditions must also be managed with appropriate medications.

The word “poly” comes from a Greek word meaning “many.” The word “chondritis” comes from a Latin word describing inflammation of the cartilage. Putting it together, the name makes sense. “Relapsing polychondritis” means inflammation of cartilaginous tissues that comes and goes.

Understanding Relapsing Polychondritis

Symptoms

Inflammation of the cartilage is the hallmark symptom of relapsing polychondritis. The severity and duration of inflammation can vary from one person to another.

Ears

The most common cartilage affected is on the top of the ears, but relapsing polychondritis spares the earlobes because there is no cartilage located there. One or both ears may become hot, red, and very sore. Some people might have difficulty laying their ear on a pillow to sleep due to the pain. Often times the inflammation of the ear cartilage can be misdiagnosed as a type of skin infection called “cellulitis.” If the inflammation does not go away after treatment with an antibiotic, chances are it’s not cellulitis. When multiple attacks affect the ear, it can sometimes make the top of the ear look a bit like cauliflower.

Nose

Relapsing polychondritis may also affect the cartilage over the bridge of the nose. The attacks can weaken the cartilage resulting in a “saddle nose” deformity, or a depression in the top of the nose that makes it look a bit like a saddle. Inflammation in the nose can lead to nose bleeds and cause crusting in the nose. Some people’s sense of smell can be affected.

Eyes

Relapsing polychondritis can have several presentations in the eye. The outside of the eye can become red and inflamed, but this is rarely painful (called episcleritis). When deeper structures of the eye are affected there can be pain (scleritis). In some cases, inflammation of the uvea (uveitis) and/or of the iris can occur.

Lungs

Cartilage is an important connective tissue in the respiratory tract. The windpipe and bronchi are made up of cartilage that holds them open while air flows in and out. When the cartilage becomes inflamed, the airways can start to collapse when you breathe out. Some people can also develop a “barking seal like” chronic cough.

Joints

The joints are made up of cartilage that acts as a cushion between bones. When joint cartilage becomes inflamed, it can be painful. One or many joints can be affected. The most common joints affected are the ones in the sternum (the breastbone). This can make it painful to breathe.

Heart

In rare cases, the valves in the heart can become inflamed. This can lead to problems with blood flow within the heart.

Like other autoimmune disorders, relapsing polychondritis can cause substantial fatigue. That’s because your immune system is ‘turned on’ all the time.

When relapsing polychondritis is associated with other autoimmune or inflammatory disorders, the signs and symptoms can be highly variable.

Diagnosis

A rheumatologist – a specialist in the treatment of arthritis and autoimmune diseases – may be required to diagnose the disease. Your rheumatologist will take a complete history and perform a thorough physical examination. This is usually followed by blood tests and other types of tests.

Blood tests can be done to look for inflammation and help diagnose viral arthritis. However, a blood test alone is not enough to diagnose the disease. If there is evidence of acute viral illness, tests to confirm the infection may be performed. There are several other tests that can help make a diagnosis:

Causes

Relapsing polychondritis is an autoimmune disease. That means for some reason, the body decides to mount an attack against its own cartilage. We don’t know why some people get relapsing polychondritis and others don’t.

Relapsing polychondritis can occur in people with other autoimmune conditions such as vasculitis and other connective tissue diseases, or with cancer. About one in three people with relapsing polychondritis has one of these other conditions. But in two in three people, there is no other condition associated with relapsing polychondritis.

Who gets Relapsing Polychondritis?

Relapsing polychondritis is very rare. This condition is more common in Caucasian people and typically affects people in their 40s to their 60s.

Relapsing polychondritis sometimes occurs before other conditions including rheumatoid arthritis, thyroid disease, inflammatory bowel disease, or myelodysplastic syndrome, a type of cancer.

Treatments

Relapsing polychondritis is a very rare disorder. Unfortunately, there is no cure for relapsing polychondritis but there are very good medications that can make living with the condition much more comfortable.

Relapsing polychondritis is a variable disease. Your treatment plan will be tailored to you and the symptoms that bother you. It will also depend on whether your relapsing polychondritis is associated with other autoimmune or inflammatory disorders.

Here are some recommendations:

  • Learn as much as you can about this disease. Knowledge is power and we’ve aimed to develop this RheumInfo website so it’s easy to understand
  • Attend your medical appointments regularly
  • Treat associated conditions such as other autoimmune or inflammatory disorders
  • Learn about the medications used to treat relapsing polychondritis. The RheumInfo website has many tools to help you understand these medications

People with relapsing polychondritis can lead active and productive lives with the right kinds of treatment. It is important to treat associated autoimmune or inflammatory disorders if they are present.

Medications for Relapsing Polychondritis

There are many medications that can be used to treat relapsing polychondritis. The choice of medications will depend on your specific symptoms.

The first line of treatment is usually Non-Steroidal Anti-Inflammatory Drugs or NSAIDs. These medications are very effective at reducing the inflammation associated with relapsing polychondritis in the ears, nose and joints. When NSAIDs are not enough to control symptoms, prednisone may help. Sometimes an antibiotic medication called Dapsone is used. When symptoms persist despite trying these medications, then methotrexate and other disease-modifying anti-rheumatic drugs (DMARDs) may be prescribed.

When the disease affects the eyes, respiratory tract, or heart, then higher doses of prednisone may be needed. Or, if you have a form of vasculitis associated with relapsing polychondritis that affects your kidneys or nerves then more aggressive treatment may be needed. Often DMARDs are used to treat these underlying conditions, such as azathioprine (Imuran), methotrexate, or cyclophosphamide (Cytoxan).

Exercises for Relapsing Polychondritis

If your joints are affected by relapsing polychondritis, physical therapy and exercise can be an important part of your overall treatment plan. Moderate stretching can help reduce pain and keep the joints flexible. Aerobic exercise such as walking, swimming or bicycling works your heart and increases your overall fitness. It is also an important part of keeping a healthy weight and can help reduce fatigue.

A trained arthritis physiotherapist can help design an exercise program tailored to you and your needs. He or she can also help you learn how to protect your joints at work and when exercising.

Read this useful article on exercising with arthritis – many of the tips can also be applied to people with relapsing polychondritis.

Surgery for Relapsing Polychondritis

When vital body systems such as the heart or respiratory tract are affected, surgery might be required. Valve replacement surgery might be needed if your heart valves are damaged by the inflammation. Stenting might be needed if you have obstructed airways due to inflammation. This is extremely rare.