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 or biologics for refractory cases or respiratory involvement. 1

Treatment Strategy Based on Disease Severity

First-Line Treatment

• Mild disease (limited to ears/nose):

  • Non-steroidal anti-inflammatory drugs
  • Short course of corticosteroids
  • Consider colchicine as background treatment 2

• Moderate to severe disease:

  • Oral prednisolone (dosage based on disease severity)
  • High-dose systemic corticosteroids for acute cases with respiratory involvement 1

Second-Line/Refractory Disease

• Conventional immunosuppressants:

  • Methotrexate
  • Azathioprine
  • Mycophenolate mofetil 2

• For severe refractory disease:

  • Rituximab (particularly effective in relapsing disease) 1
  • Cyclophosphamide (rarely used, reserved for severe cases) 2

Special Considerations for Respiratory Involvement

Respiratory tract involvement occurs in >50% of patients and significantly increases mortality 1. Early recognition and aggressive management are essential:

• Diagnostic workup:

  • Pulmonary function tests with inspiratory/expiratory flow-volume curves
  • CT scanning to detect tracheal/bronchial wall thickening, stenosis, and cartilage collapse 1
  • Consider bronchoscopy and laryngoscopy 3

• Treatment approach:

  • High-dose systemic corticosteroids for acute respiratory involvement 1
  • Early initiation of immunosuppressants to minimize corticosteroid exposure 3
  • For severe tracheobronchomalacia or stenosis:
    • Consider tracheobronchial stents or tracheostomy 4, 5
    • CPAP may be required to maintain airway patency in cases of respiratory collapse 3

Maintenance Therapy

• Long-term treatment with biologic agents (e.g., rituximab) is often required
• Recommended maintenance period of at least 18 months 1
• Aim for lowest possible dosage of corticosteroids combined with background immunosuppressive therapy 2

Monitoring

• Monitor disease activity every 1-4 weeks until remission is achieved
• Once in stable remission, extend monitoring intervals to 3-6 months 1
• For patients on long-term corticosteroids:
  • Bone densitometry
  • Osteoporosis prophylaxis 1

Prognostic Factors

• Poor prognostic factors include:
  • Respiratory tract involvement
  • Cardiovascular involvement
  • Association with myelodysplasia/VEXAS syndrome (more common in men over 50) 2, 6
  • Ethnicity, nasal chondritis, laryngotracheal stricture, and elevated serum creatinine 3

Clinical Pearls

• Diagnostic delays (average 55 weeks) are common and can lead to irreversible damage 3
• Early recognition of respiratory symptoms is crucial as they may initially be misdiagnosed as asthma 4
• Consider relapsing polychondritis in patients with recurrent respiratory symptoms and evidence of cartilage inflammation elsewhere 4
• Disease-modifying antirheumatic drugs and biologics should be considered early to minimize corticosteroid-related adverse effects 3