Relapsing polychondritis is a very rare autoimmune disorder that causes inflammation in tissues throughout the body that are made of cartilage. 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.
Relapsing polychondritis is a chronic (long-term) disease that tends to flare. It can get better for long periods of time between flares. If inflammation caused by the disorder is not controlled, it can eventually cause permanent damage to the cartilage that cannot be repaired.
The word “poly” comes from a Greek word meaning “many.” The word “chondritis” comes from a Latin word describing inflammation of the cartilage. Putting the parts of the name together, it makes sense. “Relapsing polychondritis” means inflammation of cartilaginous tissues that comes and goes.
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.
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.
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.
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.
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.
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”.
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 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
Blood tests can help to confirm the diagnosis of relapsing polychondritis. Some specific tests will look for other conditions that are commonly associated with relapsing polychondritis, such as autoimmune or inflammatory disorders.
These are some of the other tests that can help diagnose relapsing polychondritis:
- Complete blood count (CBC) with differential to look for chronic inflammation and to rule out malignancy (cancer)
- Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) – markers of inflammation
- Antinuclear antibody (ANA), Anti-neutrophil cytoplasmic antibody (ANCA), and Rheumatoid factor (RF) tests – to look for other autoimmune conditions such as vasculitis and rheumatoid arthritis
X-rays: A baseline (start) chest X-ray is often ordered so doctors can look for lung involvement.
Looking for nerve involvement: Nerve conduction studies can be performed to look for nerve involvement
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.
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.
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).
For more information about specific medications used to treat relapsing polychondritis or conditions associated with it, refer to the “pictopamphlets” in the Medications section of this website.
Living with RP
In some cases, relapsing polychondritis can be seriously interfere with your ability to work. This is particularly true during periods when the disease is flaring. The fatigue associated with relapsing polychondritis can also make it difficult to work. The good news is that once your symptoms are under control there is hope that you can resume your work.
Exercise is important to overall health. It also helps keep the joints moving properly, and helps protect them by strengthening the muscles around them.
The level and amount of exercise people with RP can do depends on the activity of their disease.
A trained arthritis physiotherapist is the best person to help design an exercise program tailored to the needs of patients with RP.
The following are some useful articles on exercising with arthritis:
Exercise and Arthritis: An article by arthritis physiotherapist Marlene Thompson
Exercising in a Flare: Another excellent article by Marlene Thompson on how to cope with flares through your exercise routine.
Many of us like to share a glass of wine, a beer or a spirit from time to time. Unfortunately, due to the nature of relapsing polychondritis, some people may turn to alcohol to help cope with the pain and the distress. Alcoholic beverages are not an effective treatment for relapsing polychondritis. They can also interact with some medications.
People with RP that plan on drinking should ask their doctor about possible negative interactions with the medications they take.
Some examples of medication that mixes poorly with alcohol includes, and is not limited to: the DMARDs methotrexate, Arava (leflunomide), and Imuran (azathioprine).
Smoking is not healthy for people with RP. It can make symptoms worse and harder to treat.
Research has shown that some arthritis medications do not work as well in people who smoke.
We recommend that smokers quit immediately to reduce the impact of their symptoms, improve the effectiveness of their medications, and improve their overall health.
Smoking significantly increases the risk of cardiovascular disease. The chronic (long-term) inflammation caused by rheumatic diseases like RP already increases this risk without a big extra boost from smoking.
Travel is still possible when you have RP.
It is best for people with this disease to get organized well in advance of a trip to ensure a smooth, comfortable, and enjoyable time.
Check out our Travel Checklist page.
Though RP does not particularly cause a loss of sex drive, it can cause pain, fatigue and emotional hardships. These hardships risk creating barriers to sexual needs, ability, and satisfaction.
People with RP can take comfort knowing that sex and intimacy can be maintained. In many cases, it can help draw partners closer together, especially through improved communication.
For more information on intimacy and arthritis, a great book is: Rheumatoid Arthritis: Plan to Win by Cheryl Koehn, Taysha Palmer and John Esdaile.
Relapsing polychondritis typically affects people in their middle to late years, so pregnancy is not usually a concern. If you have been diagnosed with relapsing polychondritis and want to get pregnant, it is important to discuss this first with your doctor.
While relapsing polychondritis itself does not interfere with breastfeeding, you should check with your doctor first. Some medications used to treat relapsing polychondritis or conditions associated with the disease can cross into breast milk.
Patients with RP might be treated with a number of medications that can affect pregnancies including NSAIDs, anti-TNF biologics, and occasionally DMARDs. It is important for people with RP who want to become pregnant to discuss their medications with their doctor and create a pre-pregnancy plan. A plan is important for a number of reasons. For example, discontinuing a particular medication for safety of the newborn might cause RP to flare, so it’s wise to have a plan in place to handle these possibilities.
Following the basics of healthy eating can help improve health and well-being in everyone, including those with RP. Keeping a healthy weight helps reduce the load on weight-bearing joints including the spine, hips, and knees, making it easier to live with the disease.
Unfortunately, no special diet has ever been proven to significantly alter the course of RP or any other types of arthritis.
In addition, no known natural remedies or complementary therapies have been proven to help RP in any significant way.
People with RP should consult with their doctor to make sure that any supplements or alternative therapies they consume will not negatively interact with their medications.
It’s very important for people with RP to attend regularly scheduled appointments with their rheumatologist, and promptly get any tests that are ordered by their doctors.
It is often very important that medications are taken exactly as prescribed. Patients should always talk to their doctor about any issues they experience, or if they would like to change anything.
Relapsing Polychondritis is a chronic disease and a rheumatologist is the best partner in helping patients achieve the best quality of life possible.