What Is Relapsing Polychondritis?
Relapsing polychondritis is a disorder in which defects develop in cartilage and other tissues throughout the body, including the ears, nose, eyes, joints, and respiratory tract. It is considered a rare condition .
Symptoms vary widely by case, but you may experience ear pain, redness, and structural changes, hearing loss or other ear impairments, eye pain, vision loss, and other eye problems, nose deformities and other nasal and respiratory issues such as trouble breathing, joint pain, chest discomfort, and full-body swelling.
Since this is a chronic condition, treatment focuses on alleviating symptoms and associated disorders.
You should visit your primary care physician for mild severity of relapsing polychondritis. Medications such as NSAIDs and steroids are generally prescribed.
How common is Relapsing Polychondritis?
Relapsing Polychondritis is also known as
- Red ear syndrome
Relapsing Polychondritis Symptoms
Several different body structures and systems will be affected in cases of relapsing polychondritis, including the ears, eyes, the nose, the respiratory system, joints, and chest [1,2].
A variety of ear-related symptoms and inflammation will likely develop, resulting in:
- Pain: This may develop suddenly or gradually, and may last for days or weeks.
- Redness: The ear may turn a red or purple color, but the ear lobe usually remains normally colored because it does not contain cartilage.
- Structural changes: These changes will likely be long-term. Repeated inflammation can cause changes to the structure of the ear, making it look floppy.
- Hearing loss, ringing in the ears, or vertigo: About one-third of people with relapsing polychondritis will eventually develop hearing loss, ringing in the ears (tinnitus), or vertigo . This can occur if the inflammation spreads to the inside of the ear to the structures responsible for hearing and balance.
About half of people with relapsing polychondritis will experience eye symptoms over the course of their disease. These symptoms include:
- Eye pain or redness
- Blurry vision
- Vision loss
- Swelling behind the eye
About half of people with relapsing polychondritis will experience nasal symptoms over the course of the disease, including :
- Stuffy nose
- Runny nose
- Bloody nose
- Impaired sense of smell
- Deformities of the nose: Repeated or prolonged inflammation can change the shape of the nose, causing a "saddle nose deformity" in which the top of the nose is caved in.
About half of people with relapsing polychondritis will develop symptoms of the upper airways, including:
- Trouble breathing
- Noisy breathing
- Voice changes
Over half of people with relapsing polychondritis will develop joint pain which can occur in :
- Both small and large joints
- One or multiple joints
- Near the chest
- The arms, legs, or back
A minority of people with relapsing polychondritis may develop disease of the heart valves, which can cause heart failure. This may cause symptoms such as:
- Chest discomfort
- Trouble breathing
- Swelling in the abdomen or legs
Relapsing Polychondritis Causes
The cause of relapsing polychondritis is unknown, but some factors are thought to contribute to the development of relapsing polychondritis. These factors include genetic mutations, other immune system disorders, recreational drug use, and infections.
Certain genetic mutations are thought to increase the risk of developing relapsing polychondritis. These include mutations in genes known as "human leukocyte antigens," which are proteins found on certain cells of the immune system .
Other immune system disorders
Relapsing polychondritis is often associated with other disorders of the immune system. It is believed that these immune system disorders may create an imbalance that causes the immune system to attack the body's own cartilage and tissues. These include disorders known as :
- Vasculitis: Inflammation of blood vessels
- Graves' disease: An autoimmune disease of the thyroid gland
- Ulcerative colitis: An autoimmune disease of the gastrointestinal tract
Recreational drug use
Cases of relapsing polychondritis have been reported to occur after episodes of recreational drug use . The drug or other chemicals mixed into the drugs may either directly damage the cartilage tissue, or create an imbalance in the immune system that causes damage to the cartilage tissue.
Some infections may contribute to the development of relapsing polychondritis by either directly damaging cartilage tissue, or by triggering the immune system to attack the body's own cartilage.
Treatment Options and Prevention
Relapsing polychondritis is a chronic disorder that cannot be completely cured [1,8]. Therefore, treatment is focused on reducing symptoms and treating associated disorders. Specific treatment options include anti-inflammatory medications, steroid injections for joint pain, vaccinations, surgery to treat airway or heart problems, and surgery to repair deformities in your appearance.
This is the primary form of medical treatment for relapsing polychondritis. The specific medication your physician may recommend will depend on the severity of symptoms and your response to other medications.
- NSAIDs: People with mild or moderate disease without involvement of internal organs will usually be treated with nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen (Advil) or naproxen (Aleve).
- Oral or topical steroids: People who do not respond to NSAIDs may be started on steroids such as prednisone or another anti-inflammatory medication called dapsone (Aczone).
- Other anti-inflammatories: People who do not respond either NSAIDs or steroids may be started on other anti-inflammatory medications including methotrexate, azathioprine (Imuran), cyclophosphamide or cyclosporine, among others.
Steroid injections for joint pain
People who are experiencing joint pain may benefit from steroid injections to the inflamed joints. These may be repeated at regular intervals until the inflammation subsides.
People with relapsing polychondritis are at an increased risk of developing respiratory infections, both because they may have disease in the upper airways and because they are frequently on immunosuppression medications. Therefore, your physician may recommend receiving vaccinations such as the flu and pneumococcal vaccines.
Surgery to treat airway or heart problems
For associated airway and heart problems, you may undergo the following surgeries:
- A stent: This will be placed in the airways to keep them open.
- A tracheostomy: This will be placedin the front of the neck to assist with breathing.
- Reconstruction: If the airways collapse, reconstruction may be needed.
- Heart surgery: This will be done to address any damage and to repair valves.
Surgery to repair deformities in the ears or nose
These surgeries are usually only done once the active inflammation has subsided. If the inflammation is not well-controlled after the surgery, the deformities may recur.
When to Seek Further Consultation
You should seek medical attention if you have reason to believe you have relapsing polychondritis or you have trouble breathing.
If you develop any symptoms of relapsing polychondritis
You should go see your physician if you experience inflammation of the ears, nose, or eyes, trouble breathing, or joint pain.
If you are having trouble breathing
You should consider going directly to the emergency room if you are experiencing trouble breathing. Your physician can do an examination and order testing to determine if you have relapsing polychondritis as well as any disorders that are classically associated with relapsing polychondritis, and subsequently offer the appropriate treatment.
Questions Your Doctor May Ask to Diagnose
To diagnose this condition, your doctor would likely ask about the following symptoms and risk factors.
- Any fever today or during the last week?
- Have you been feeling more tired than usual, lethargic or fatigued despite sleeping a normal amount?
- Do you have a cough?
- Have you lost your appetite recently?
- Have you experienced any nausea?
The above questions are also covered by our A.I. Health Assistant.
- Relapsing Polychondritis. National Institutes of Health: Genetic and Rare Diseases Information Center (GARD). Published April 21, 2015. NIH Link
- Relapsing Polychondritis. National Organization for Rare Disorders. NORD Link
- Emmungil H, Aydin SZ. Relapsing Polychondritis. European Journal of Rheumatology. 2015;2(4):155-159. EJR Link.
- Sharma A. Relapsing Polychondritis. Orphanet. Published July 2014. Orphanet Link
- Terao C, Yoshifuji H, Yamano Y, et al. Genotyping of Relapsing Polychondritis Identified Novel Susceptible HLA Alleles and Distinct Genetic Characteristics From Other Rheumatic Diseases. Rheumatology (Oxford). 2016;55(9):1686-1692. PubMed Link
- Caspary L. Vasculitides of Large Vessels. Journal of Vascular Diseases. 2011;40(2):89-98. JVD Link
- Michet CJ, Buckner JH, Romain PL. Etiology and Pathogenesis of Relapsing Polychondritis. UpToDate. Published July 2, 2018. UpToDate Link
- Lekpa FK, Chevalier X. Refractory Relapsing Polychondritis: Challenges and Solutions. Open Access Rheumatology. 2018;10:1-11. DovePress Link