Relapsing Polychondritis

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

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

Ears

The most common cartilage affected is on the top of the ears. 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, 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.

Joints

The joints are made up of cartilage that acts as a cushion between bones. It can be painful when joint cartilage becomes inflamed. 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.

Respiratory Tract

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 during breaths out. Some people can also develop a chronic cough that is sometimes compared to the sound of a “barking seal”.

Heart

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

Fatigue

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

Relapsing Polychondritis (RP) is best diagnosed by a rheumatologist, a type of doctor that specializes in arthritis, autoimmune, and autoinflammatory diseases. RP can be tricky to diagnose and having someone experienced is very useful.

To diagnose RP, a rheumatologist will take a careful and complete history and perform a thorough physical examination. The doctor will be seeking to confirm that their patient meets a “checklist” of characteristic signs and symptoms associated with Relapsing Polychondritis.

Next, the doctor will usually order blood tests, x-rays, and other types of tests to confirm their diagnosis and rule out other possible conditions.

Common Tests for Relapsing Polychondritis

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.

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.

NSAIDs

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.

Antibiotic Treatment

Sometimes an antibiotic medication called Dapsone is used.

DMARDs

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.

In people that have a form of vasculitis associated with relapsing polychondritis that affects the kidneys or nerves, a more aggressive treatment may be needed. Often DMARDs are used to treat these underlying conditions, such as azathioprine (Imuran), methotrexate, or cyclosporine (Cytoxan).