Relapsing Polychondritis (RP) is a rare, systemic autoimmune disorder that primarily targets cartilaginous and proteoglycan-rich tissues throughout the body. A recent case report involving a 24-year-old male highlights how this condition often mimics common infections or localized inflammation. Consequently, diagnostic delays are frequent, which can lead to irreversible damage such as permanent sensorineural hearing loss. Clinicians must maintain a high index of suspicion when patients present with recurrent ocular, nasal, or auricular symptoms.

Understanding the Diagnostic Challenge of Relapsing Polychondritis

Diagnosis of Relapsing Polychondritis remains largely clinical because no specific laboratory markers exist. The McAdam criteria, often supplemented by the Damiani and Michet criteria, require the presence of at least three clinical features. These include recurrent auricular chondritis, non-erosive inflammatory polyarthritis, nasal chondritis, ocular inflammation, respiratory tract chondritis, and audiovestibular damage. In this case, the patient exhibited multiple symptoms across these categories over time. However, the initial episodic nature of the symptoms meant he received only short-term corticosteroid treatments, which masked the underlying systemic disease.

Clinical Presentation and Permanent Sequelae

The patient initially experienced ocular redness and nasal discharge, followed by septal perforation. Later, he developed bilateral aural perichondritis, which typically spares the earlobes—a hallmark sign of Relapsing Polychondritis. The most devastating complication in this instance was the sudden onset of severe bilateral sensorineural hearing loss. While corticosteroids effectively manage acute inflammatory flares, they did not prevent the progression to permanent audiovestibular damage in this patient. This underscores the necessity for aggressive, long-term immunosuppressive management in severe cases.

Therapeutic Strategies for Relapsing Polychondritis

Effective management depends on the severity and specific organs involved. For mild auricular or nasal involvement, NSAIDs or low-dose corticosteroids may suffice. Conversely, severe manifestations like hearing loss or airway involvement require high-dose intravenous methylprednisolone. Furthermore, steroid-sparing agents like methotrexate, azathioprine, or cyclophosphamide are often necessary to maintain remission. Emerging evidence also suggests a role for biologic therapies, including TNF-alpha inhibitors, for patients refractory to conventional treatments.

FAQs

What are the primary diagnostic criteria for relapsing polychondritis?

Diagnosis typically follows the McAdam criteria, requiring three or more features: bilateral auricular chondritis, non-erosive inflammatory arthritis, nasal chondritis, ocular inflammation, respiratory tract chondritis, and audiovestibular dysfunction. The Damiani and Michet criteria are also used to support diagnosis when fewer clinical features are present alongside positive histology or treatment response.

Why is hearing loss a significant concern in relapsing polychondritis?

Hearing loss in RP is often sensorineural and can occur suddenly due to vasculitis of the internal auditory artery or inflammation of the labyrinth. Because this damage is frequently irreversible, prompt recognition and intensive anti-inflammatory treatment are critical to preserve auditory function.

How is relapsing polychondritis managed clinically?

Management is tailored to disease severity. While mild cases are treated with NSAIDs and low-dose steroids, severe or progressive disease requires high-dose corticosteroids and immunosuppressants like methotrexate or cyclophosphamide. Biologic agents are considered for refractory cases.

Disclaimer: This content is for informational and educational purposes only. It does not constitute professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified healthcare provider with any questions you may have regarding a medical condition. Refer to the latest local and national guidelines for clinical practice.

References

Dari MA et al. A case of relapsing polychondritis: a diagnostic challenge in recurrent cartilaginous inflammation and hearing loss-A case report. J Med Case Rep. 2026 Apr 12. doi: 10.1186/s13256-026-05977-1. PMID: 41968374.

Lekpa FK, Chevalier X. Relapsing polychondritis. RMD Open. 2018;4(Suppl 1):e000788. doi: 10.1136/rmdopen-2018-000788.

Borgia F, et al. Relapsing Polychondritis: An Updated Review. Biomedicines. 2018; 6(3):84. doi: 10.3390/biomedicines6030084.