Standard of Care Treatment Strategies

Because relapsing polychondritis (RP) is so rare, there are currently no formal “gold standard” international clinical practice guidelines for its treatment. Instead, your care team will rely on expert consensus, clinical experience, and observational studies to create a plan tailored to you ^[1][2].

The primary goal of treatment is twofold: to quickly quiet the inflammation to prevent permanent damage to your cartilage and organs, and to find the lowest effective dose of medication to minimize long-term side effects ^[3][4].

An Individualized Approach

Doctors tailor treatment based on disease severity and which organs are involved, recognizing that apparently limited ear/nose disease can sometimes coexist with occult airway or vascular involvement ^[4][5].

• Minor or Limited Disease: If your symptoms are restricted to your outer ears or nose and do not involve your breathing, eyes, heart, or nervous system, the focus may be on symptom control. This may involve NSAIDs (nonsteroidal anti-inflammatory drugs like ibuprofen), colchicine, or a short, temporary course of low-dose corticosteroids ^[4][5].
• Moderate to Severe (Organ-Threatening) Disease: If the disease affects your airways, eyes, nervous system, or heart, or if it keeps returning, urgent specialist assessment and more powerful medications are needed. The foundation of treatment is usually systemic corticosteroids (like prednisone) ^[6][7]. While these work quickly, they can cause significant side effects over time, so your doctor will aim to “taper” the dose as soon as it is safe to do so ^[8]. Never stop prednisone abruptly without medical guidance. Discuss bone protection, blood pressure, glucose monitoring, and eye monitoring with your clinician.

Steroid-Sparing Agents

To help you reduce the amount of prednisone you need, doctors often add conventional immunosuppressants. These are sometimes called “steroid-sparing agents” because they allow the body to maintain control over the disease even as the steroid dose is lowered ^[5].

Commonly used options include:

• Methotrexate: One of the most frequently used agents, shown in observational studies to help many patients ^[1].
• Azathioprine or Mycophenolate Mofetil: These are often used if methotrexate is not tolerated or is not effective enough ^[5][6].
• Cyclophosphamide: This is a very potent medication typically reserved for life-threatening situations, such as severe airway narrowing or inflammation of the central nervous system ^[5][9].

Escalation to Biologic Therapy

If conventional treatments do not work, or if your disease is particularly aggressive, your doctor may recommend biologics. These are engineered proteins that target very specific parts of the immune system ^[6]. Data on biologics comes primarily from small observational series, so their use is generally specialist-directed.

• TNF Inhibitors (e.g., Infliximab, Adalimumab): These block a protein called tumor necrosis factor that drives inflammation ^[1].
• IL-6 Inhibitors (e.g., Tocilizumab): This targets a different inflammatory pathway and has been noted in case series for helping with refractory ocular and heart involvement in some patients ^[1][10][11].

Managing the Risks of Treatment

All treatments that suppress the immune system come with a trade-off. The most common concern is an increased risk of infection ^[2]. In a large study of patients on biologics for RP, infections were the most frequently reported side effect ^[2].

Your care team will monitor you closely with regular blood tests to check your liver and kidney function and your white blood cell counts ^[12]. Because the course of RP can be unpredictable, your treatment is not set in stone; it will be clinician-directed to “step up” during active flares and “step down” carefully when you are in remission ^[13][14]. Discuss vaccination timing with your doctor, as live vaccines are typically contraindicated while immunosuppressed.