Understanding Relapsing Polychondritis: Symptoms, Causes, and Care

Medically reviewed by Dr. Baraa Alnahhal, MD · Last reviewed: October 2026

• Definition: A rare, chronic disorder with recurring inflammation and eventual deterioration of cartilage throughout the body.

• Main targets: The ears, nose, and airway, often presenting with redness, swelling, and pain.

• Cause: The exact cause is unknown, but it is widely considered an autoimmune condition.

• Treatment: Anti-inflammatory and immunosuppressive medications manage episodes, while severe cases may need surgery to protect the airway or heart valves.

Quick Answer

Relapsing polychondritis is a rare autoimmune-related disorder that causes repeated episodes of cartilage inflammation and breakdown, most commonly in the ears, nose and respiratory tract. Symptoms include ear redness, nasal congestion and joint pain. There is no cure, but corticosteroids and immunosuppressants can reduce the intensity and frequency of flares and help manage the condition. ^[1]

What the research says

• Treatment is based on observational data, not randomized trials. A systematic review of 11 studies (177 patients, 247 treatment lines) found no randomized trial has been done. Pooled response rates were 72% for abatacept (95% CI 42 to 95), 66% for tocilizumab (49 to 82), 64% for TNF inhibitors (53 to 74), and 56% for methotrexate (37 to 73); methotrexate had the most robust data (Petitdemange et al., Clin Exp Rheumatol, 2022).

• Some people diagnosed with relapsing polychondritis have a different disease. A clinical review reports that VEXAS syndrome, caused by UBA1 gene mutations, accounts for about 8% of patients with a clinical diagnosis of relapsing polychondritis, and that diagnosis is hard because symptoms fluctuate and there are no specific markers or universally accepted classification criteria (Mertz et al., Best Pract Res Clin Rheumatol, 2023).

What is relapsing polychondritis?

Relapsing polychondritis is a complex and rare disorder that leads to the inflammation and deterioration of cartilage, the essential connective tissue found throughout the human body. The condition is characterized by its recurring nature, where patients experience flares or episodes that can last for days or weeks. These episodes often repeat over several years, leading to progressive damage if left unmanaged.

This condition is extremely rare, with researchers estimating it affects approximately 1 in 285,000 people. While it can affect individuals of any age, it most commonly manifests in people between the ages of 40 and 60. In medical literature, the disorder is also known by several other names, including chronic atrophic polychondritis, Von Meyenburg disease, and systemic chondromalacia. ^[1]

Feature	Details
Prevalence	Approximately 1 in 285,000 people
Primary Targets	Ears, nose, larynx, and trachea
Common Age of Onset	40 to 60 years old
Survival Rate	~95% alive eight years post-diagnosis

What are the symptoms of relapsing polychondritis?

The initial symptoms of relapsing polychondritis typically involve the ears. Patients often report sudden pain, tenderness, swelling, and redness in one or both ears. Notably, this inflammation affects the cartilage of the ear but spares the ear lobes, which do not contain cartilage. Over time, repeated flares can cause the ears to lose their structural integrity and become floppy.

As the condition progresses, it may affect other cartilage-rich areas of the body. Cartilage collapse at the bridge of the nose can result in a visible "saddle nose" deformity. When the respiratory tract is involved, patients may experience difficulty breathing or speaking. In rare but severe cases, the inflammation can extend to the eyes, heart valves, and kidneys, potentially leading to vision loss or life-threatening heart complications. ^[1]

Figure 1: Primary cartilage sites affected by relapsing polychondritis.

What causes relapsing polychondritis and why is it hard to diagnose?

The exact cause of relapsing polychondritis remains unknown to modern science. Many experts classify it as an autoimmune disease, a state where the body's immune system mistakenly attacks its own healthy connective tissue. However, the specific triggers that initiate this immune response are not yet understood, and there are currently no known strategies to prevent the condition.

Diagnosing relapsing polychondritis is particularly challenging because there is no single definitive test for the disorder. Healthcare providers typically reach a diagnosis based on a clinical evaluation of the patient's symptoms over time. A diagnosis is generally confirmed if a patient exhibits at least three of the associated symptoms. In some instances, a biopsy of the ear cartilage or specific blood tests may be used to support the clinical findings. ^[1]

Figure 2: Clinical checklist for identifying relapsing polychondritis.

How is relapsing polychondritis managed and treated?

Since there is no cure for relapsing polychondritis, the primary goal of treatment is to ease current symptoms and reduce the risk of long-term complications. For mild to moderate flares, providers often prescribe nonsteroidal anti-inflammatory drugs (NSAIDs), corticosteroids like prednisone, or medications such as Dapsone and Colchicine.

In more severe or persistent cases, immunosuppressive therapy may be required. This can include medications like methotrexate, azathioprine, or cyclophosphamide, which work to dampen the overactive immune response. For patients facing life-threatening complications, such as a collapsing airway or heart valve failure, surgical interventions like tracheostomies, stent insertions, or valve replacements may be necessary to maintain vital functions. ^[1]

Figure 3: Treatment progression pathway for relapsing polychondritis.

Conclusion and Next Steps

Relapsing polychondritis is a lifelong chronic condition that requires ongoing medical supervision. While the rarity of the disorder can make the initial diagnosis difficult, most patients can maintain a good quality of life with proper management. By working closely with specialists to control flares and monitor for respiratory or cardiovascular issues, individuals can significantly reduce the risk of permanent damage and life-threatening complications.

Frequently Asked Questions

Is relapsing polychondritis an autoimmune disease?

Yes, many scientists consider relapsing polychondritis to be an autoimmune disease. This occurs when the immune system incorrectly identifies healthy cartilage as a threat and attacks it, leading to chronic inflammation.

Can relapsing polychondritis affect hearing?

Yes, if the inflammation affects the inner ear, it can lead to hearing loss, tinnitus (ringing in the ears), or vertigo (a spinning sensation). Prompt medical attention is recommended if these symptoms occur.

What is "saddle nose" deformity?

A saddle nose deformity occurs when the cartilage at the bridge of the nose collapses due to inflammation from relapsing polychondritis. This results in a visible indentation or flattening of the nasal bridge.

What are the first signs of a relapsing polychondritis flare?

The most common early signs are pain, redness, and swelling in the ears. Unlike other infections, this redness typically affects only the cartilage and does not involve the ear lobes.

How is relapsing polychondritis diagnosed?

There is no specific test for the condition. Diagnosis is usually based on a clinical history of at least three associated symptoms. Doctors may also use biopsies or blood tests to confirm the diagnosis.

Is relapsing polychondritis fatal?

While most people have a good outlook with a high survival rate, the condition can be life-threatening if it causes severe breathing problems or heart valve complications.

What medications are used for relapsing polychondritis?

Common treatments include NSAIDs, corticosteroids (like prednisone), and immunosuppressants (like methotrexate or azathioprine) to control inflammation and prevent flares.

Can children get relapsing polychondritis?

While the condition can affect anyone, it most commonly begins in adults between the ages of 40 and 60. It is considered very rare in children.

Does relapsing polychondritis go away?

No, it is a chronic condition that does not have a cure. However, with consistent medical treatment, the frequency and intensity of flares can be significantly reduced.

References

1. Cleveland Clinic. Relapsing Polychondritis. Cleveland Clinic Health Library. Accessed October 11, 2026. https://my.clevelandclinic.org/health/diseases/24846-relapsing-polychondritis

2. Petitdemange A, Sztejkowski C, Damian L, et al. Treatment of relapsing polychondritis: a systematic review. Clin Exp Rheumatol. 2022;40 Suppl 134(5):81-85. PMID: 35238756. DOI: https://doi.org/10.55563/clinexprheumatol/h9gq1o

3. Mertz P, Sparks J, Kobrin D, et al. Relapsing polychondritis: Best Practice & Clinical Rheumatology. Best Pract Res Clin Rheumatol. 2023;37(1):101867. PMID: 37839908. DOI: https://doi.org/10.1016/j.berh.2023.101867

Medical disclaimer

This article is for informational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have about a medical condition.