Short answer · Medically reviewed summary · Last updated: 2026-05-08
Relapsing polychondritis is a rare, systemic autoimmune condition characterized by recurrent inflammation and destruction of cartilage throughout the body, most notably in the ears, nose, and respiratory tract. Because there is no single diagnostic test for relapsing polychondritis, medical professionals rely on identifying patterns of clinical symptoms and inflammatory markers to confirm a diagnosis. What body systems are affected by relapsing polychondritis? Relapsing polychondritis is a systemic disease, meaning it can impact multiple organ systems beyond the skeletal and connective tissues.
6 people with Relapsing Polychondritis have shared their first-person experience on this question at DiseaseMaps.
What is Relapsing Polychondritis
What is Relapsing Polychondritis? Plain-language, medically reviewed definition plus the lived reality told by patients.
Relapsing polychondritis is a rare, systemic autoimmune condition characterized by recurrent inflammation and destruction of cartilage throughout the body, most notably in the ears, nose, and respiratory tract. Because there is no single diagnostic test for relapsing polychondritis, medical professionals rely on identifying patterns of clinical symptoms and inflammatory markers to confirm a diagnosis.
What body systems are affected by relapsing polychondritis?
Relapsing polychondritis is a systemic disease, meaning it can impact multiple organ systems beyond the skeletal and connective tissues. While it primarily targets cartilage (chondritis), it frequently involves the eyes, inner ear (vestibular system), and the cardiovascular system. In severe cases, inflammation of the airway cartilage can lead to structural damage that requires surgical intervention. The immune system is the primary driver of this damage, as it mistakenly attacks the body’s own healthy cartilage tissues.
Who is typically affected by relapsing polychondritis?
Relapsing polychondritis is an exceptionally rare disorder with an estimated prevalence of approximately 3.5 to 4.5 cases per million people. While it can occur at any age, the onset of relapsing polychondritis is most common between the ages of 40 and 60. Current clinical data suggests that men and women are affected in roughly equal proportions, and there is no strong evidence linking the condition to specific geographic regions or environmental factors.
What are the key clinical features of this condition?
To help guide your conversations with a rheumatologist, keep in mind that a diagnosis often requires the presence of at least three of the following characteristic features:
- Inflammation of the cartilage in both ears.
- Seronegative polyarthritis affecting five or more joints simultaneously.
- Inflammation of the nasal cartilage.
- Ocular inflammation, such as scleritis or uveitis.
- Vestibular dysfunction, including vertigo or unexplained hearing loss.
- Inflammation of the cartilage within the airway (trachea or bronchi).
How does relapsing polychondritis differ from other conditions?
Unlike rheumatoid arthritis, relapsing polychondritis is specifically "seronegative," meaning patients typically test negative for rheumatoid factor. Furthermore, while many autoimmune diseases focus on joints, relapsing polychondritis is unique in its tendency to cause structural dissolution of the cartilage in the ears and nose, often leading to a "saddle nose" deformity or "cauliflower ear" appearance if left untreated.
Next steps
- Consult a board-certified rheumatologist to discuss your specific symptoms and potential treatment plans.
- Connect with the 468 members of the DiseaseMaps.org community who share experiences with this rare condition.
- Monitor for any changes in breathing or vision, as these require urgent medical evaluation.
Medical disclaimer: This information is for educational purposes only and should not replace professional medical advice, diagnosis, or treatment.
References
- NIH Genetic and Rare Diseases Information Center (GARD): Relapsing Polychondritis.
- Orphanet: Rare disease database for Relapsing Polychondritis.
- OMIM (Online Mendelian Inheritance in Man): Clinical summary for Polychondritis, Relapsing.
- PubMed/NCBI: Clinical reviews on the management of systemic chondromalacia.
The exact cause of Relapsing Polychondritis (RP) is not known. However, it is believed to be an autoimmune disorder where the body's immune system starts attacking its own healthy cells believing them to be foreign.
RP has an episodic pattern with periods of intense inflammation and periods of remission and dependant on the severity of the inflammatory process, often requiring the use of immune suppressant medications.
RP can be life threatening, debilitating and difficult to diagnose and prognosis is variable, depending on organ involvement and treatment complications. Where the disease is mild or responds quickly to treatment, there is a good prognosis.
Relapsing Polychondritis is also known by other names such as Meyenburg Altherr Uehlinger syndrome, chronic atrophic polychondritis and Von Meyenburg syndrome.
Posted Mar 20, 2018 by Lisa Matthews (Lissy) 4800
Posted Jun 12, 2019 by Tmrcarlson 3550
Posted Jun 13, 2019 by Kaz 3000
Posted Dec 30, 2019 by Leanne 2500
Posted Dec 30, 2019 by Carrie 3050
Posted Mar 20, 2022 by shlawver 2500
