Treatment of Relapsing Polychondritis
The treatment of relapsing polychondritis requires systemic corticosteroids as first-line therapy, 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. Key features include:
- Equal male/female ratio, with mean age at diagnosis of 51 years
- Most common symptoms: auricular chondritis (88%) and arthralgias (81%)
- Relapsing course in 86% of patients
- Respiratory system involvement in >50% of patients, presenting as the initial feature in 25%
Treatment Algorithm
First-Line Treatment
- Mild disease (limited to ears or nose):
Second-Line Treatment (Moderate Disease)
- Systemic corticosteroids (prednisone) - gold standard treatment 3
- Required for most patients with significant inflammation
- Dosage based on severity of symptoms
Third-Line Treatment (Severe/Refractory Disease)
- Conventional immunosuppressants 2, 3:
- Methotrexate (5-10 mg/week)
- Azathioprine
- Mycophenolate mofetil
- Cyclophosphamide (rarely, for severe cases)
Fourth-Line Treatment (Biologic Therapy)
For patients failing conventional immunosuppressants 3:
- TNF-α inhibitors (most evidence):
- Infliximab and adalimumab preferred
- Partial or complete response in many cases
- Note: efficacy may diminish over time
- Alternative biologics:
- Abatacept or tocilizumab (effective second-line biologics)
- Anakinra and rituximab (controversial data, not recommended as first-line biologics)
- JAK inhibitors (limited evidence)
Special Considerations
Respiratory Involvement
- Present in >50% of patients and associated with increased mortality 1
- Management approach:
- Requires long-term systemic corticosteroids and immunosuppressants 1
- Pulmonary function tests with flow-volume curves to identify subtle airway involvement
- CT scanning to assess tracheobronchial wall thickening and stenosis
- Consider endobronchial stents or tracheostomy for severe stenosis refractory to medical therapy 4
Monitoring
- Regular assessment of treatment response
- Monitoring for medication side effects
- Evaluation for progression of cartilage damage
- CT scanning to assess airway involvement when respiratory symptoms present
Clinical Pearls and Pitfalls
- Key pitfall: Delayed diagnosis due to variable presentation and rarity of disease
- Warning sign: Persistent or chronic cough may be the first sign of respiratory involvement 1
- Mortality risk: Death is frequently related to respiratory complications 1
- Treatment challenge: No randomized controlled trials exist for therapy 4
- Monitoring: Fast CT scanners can visualize dynamic airway collapse in respiratory involvement 4
The treatment approach should be guided by disease severity, with particular attention to respiratory involvement which significantly impacts mortality and requires aggressive immunosuppressive therapy.