What is the treatment for relapsing polychondritis?

Treatment of Relapsing Polychondritis

Systemic corticosteroids are the first-line treatment for relapsing polychondritis, with escalation to immunosuppressants for refractory cases or respiratory involvement, which is critical for reducing mortality. 1

Disease Overview

Relapsing polychondritis is an uncommon autoimmune disorder characterized by recurrent inflammation and widespread destruction of cartilage and connective tissues. It affects men and women equally, with a mean age at diagnosis of 51 years. The most common symptoms include swelling and erythema of the ears (88%) and arthralgias (81%). The disease follows a relapsing course in 86% of patients, with continuous symptoms in 14%. 2

Treatment Algorithm

First-Line Therapy

• Systemic corticosteroids: The gold standard treatment for most patients with significant inflammation
  • Typically oral prednisolone with dosage based on disease severity
  • For acute cases with respiratory involvement, high-dose systemic corticosteroids are required 2

Second-Line Therapy (for refractory cases or respiratory involvement)

• Conventional immunosuppressants:
  • Methotrexate (for non-organ threatening disease and maintenance)
  • Azathioprine
  • Mycophenolate mofetil
  • Cyclophosphamide (for severe, organ-threatening disease) 1

Third-Line Therapy (after failure of conventional treatments)

• Biologic agents:
  • TNF-α inhibitors: First choice among biologics
    • Infliximab and adalimumab are preferred, with partial or complete response in many cases 3
  • Rituximab: Anti-CD20 antibody, particularly effective in relapsing disease 2
  • Tocilizumab: IL-6 receptor antagonist, effective as second-line biologic agent
  • Abatacept: Effective but associated with infection risk 3

Special Considerations for Respiratory Involvement

Respiratory involvement occurs in >50% of patients with relapsing polychondritis and is associated with increased mortality. It requires aggressive treatment: 2, 1

• Long-term use of systemic corticosteroids and immunosuppressants is typically required 2
• Monitoring with pulmonary function tests, including inspiratory and expiratory flow-volume curves 2
• CT scanning to assess tracheobronchial wall thickening and stenosis 2
• In severe cases of tracheobronchomalacia, airway stenting may be necessary 4, 5

Treatment Monitoring

• Disease activity should be monitored regularly, as frequently as every 1-4 weeks until remission is achieved 2
• Once in stable remission, monitoring intervals can be extended to 3-6 months 2
• Long-term treatment is often required, with a recommended maintenance period of at least 18 months 2

Prognosis and Complications

• Without treatment, relapsing polychondritis with respiratory involvement has high mortality 2
• Early diagnosis and prompt treatment are essential to decrease the risk of life-threatening airway collapse 4
• Long-term corticosteroid use requires bone densitometry and prophylaxis against osteoporosis 2
• Mortality is frequently related to respiratory complications 2, 6

Pitfalls and Caveats

• Diagnostic delay is common (average 55 weeks), which can lead to irreversible cartilage destruction 6
• Respiratory symptoms may initially be misdiagnosed as asthma 4, 5
• Persistent or chronic cough may be the first sign of respiratory involvement and should prompt evaluation 2
• Loss of efficacy with biologic agents may occur over time, requiring switching between different biologics 3
• Infections are a significant risk with immunosuppressive therapy, particularly with abatacept 3

Early, aggressive immunosuppressive treatment is crucial to prevent irreversible cartilaginous destruction and airway collapse in patients with relapsing polychondritis, particularly those with respiratory involvement.