Which are the symptoms of Relapsing Polychondritis?

Relapsing polychondritis is a rare, multisystem autoimmune disorder characterized by recurrent episodes of inflammation in cartilaginous tissues, most commonly affecting the ears, nose, and airways. Diagnosis is clinical, typically requiring a combination of symptoms such as chondritis (cartilage inflammation), polyarthritis, and ocular or vestibular involvement to confirm the condition.

What are the primary symptoms of Relapsing Polychondritis?

The clinical presentation of Relapsing Polychondritis is highly variable, as the disease can impact any cartilage-containing structure in the body. The 468 members of the DiseaseMaps community often report that symptoms appear in flares followed by periods of relative remission. Common manifestations include:

• Auricular Chondritis: Inflammation of the external ear (excluding the lobe), which appears red, swollen, and painful.


• Nasal Chondritis: Tenderness or inflammation of the nasal cartilage, which may eventually lead to a "saddle-nose" deformity.


• Polyarthritis: Pain and swelling in five or more joints, occurring in a non-erosive pattern.


• Ocular Inflammation: Redness and pain in the eyes, specifically scleritis, episcleritis, or uveitis.


• Airway Involvement: Hoarseness, cough, or difficulty breathing, indicating inflammation of the tracheal or bronchial cartilage.

What are the early warning signs to watch for?

Early signs of Relapsing Polychondritis often start with unexplained, persistent redness and tenderness of the ears. Patients should also monitor for unexplained joint pain, persistent red eyes that do not respond to standard drops, or a sudden onset of vertigo or ringing in the ears (tinnitus). Because Relapsing Polychondritis is systemic, systemic symptoms like fatigue and low-grade fever are also frequently reported during early flare-ups.

When should I seek immediate medical attention?

You must seek emergency care if you experience symptoms involving the airway, such as shortness of breath, stridor (a high-pitched wheezing sound), or significant hoarseness. Airway involvement in Relapsing Polychondritis can lead to life-threatening narrowing of the trachea and requires urgent evaluation by a rheumatologist or pulmonologist.

How does the disease progress over time?

The course of Relapsing Polychondritis is unpredictable. While some individuals experience mild, intermittent flares, others may face more frequent, severe episodes that damage cartilage structures. Over time, chronic inflammation in Relapsing Polychondritis can lead to functional impairment, such as hearing loss or respiratory issues, which is why early and consistent management is critical for long-term quality of life.

Next steps

• Consult a board-certified rheumatologist to establish a long-term treatment plan.


• Join the DiseaseMaps.org community to connect with other patients managing Relapsing Polychondritis.


• Keep a symptom diary to help your physician identify potential triggers for your specific flares.

Medical disclaimer: This information is for educational purposes only and does not replace professional medical advice, diagnosis, or treatment; always seek the advice of your physician regarding any medical condition.

References

• NIH Genetic and Rare Diseases Information Center (GARD): Relapsing Polychondritis.


• Orphanet: Relapsing Polychondritis (ORPHA:732).


• OMIM (Online Mendelian Inheritance in Man): Polychondritis, Relapsing.


• The Relapsing Polychondritis Foundation.