What is the treatment approach for a patient with relapsing polychondritis?

Treatment Approach for Relapsing Polychondritis

For patients with relapsing polychondritis, treatment should be stratified by disease severity: mild disease (isolated auricular/nasal chondritis or arthritis) can be managed with NSAIDs or short-course corticosteroids with colchicine as background therapy, while moderate-to-severe disease—particularly with respiratory involvement—requires long-term systemic corticosteroids combined with conventional immunosuppressants (methotrexate, azathioprine, or mycophenolate mofetil) or biologic therapies, with early aggressive immunosuppression potentially preventing irreversible cartilaginous destruction and airway collapse. 1, 2, 3

Disease Severity Stratification and Initial Assessment

Before initiating therapy, assess for respiratory involvement, which occurs in >50% of patients and represents the most serious manifestation responsible for mortality 1:

• Perform pulmonary function tests with inspiratory and expiratory flow-volume curves to identify clinically subtle airway involvement, even when symptoms are minimal 1
• Obtain CT scanning to evaluate for tracheobronchial wall thickening, stenosis, cartilage collapse and calcification, or bronchiectasis in late-stage disease 1
• Consider bronchoscopy if imaging is inconclusive or to directly visualize inflammation, collapse, and stenosis of the larynx, trachea, and/or bronchi 1
• Recognize that persistent or chronic cough is often one of the first and most important symptoms signifying respiratory involvement 1

Treatment Algorithm by Disease Severity

Mild Disease (Isolated Auricular/Nasal Chondritis or Peripheral Arthritis)

For patients without significant organ involvement 3, 4:

• Initiate NSAIDs or salicylates as first-line therapy for symptomatic relief 1
• Add short courses of low-dose corticosteroids (specific dosing not defined in guidelines, but typically prednisone 10-20 mg daily) for acute flares 3, 4
• Consider colchicine as background therapy to reduce relapse frequency and minimize corticosteroid exposure 3

Moderate-to-Severe Disease (Respiratory, Cardiovascular, or Multi-Organ Involvement)

Respiratory involvement mandates aggressive immunosuppression because it is the primary cause of mortality and requires long-term treatment 1, 5:

• Start systemic corticosteroids at the lowest effective dose (typically prednisone 0.5-1 mg/kg/day for induction, though specific dosing not codified) 1, 3
• Combine with conventional immunosuppressants as steroid-sparing agents 3:
  • Methotrexate (preferred first-line conventional agent)
  • Azathioprine
  • Mycophenolate mofetil
  • Cyclophosphamide (reserved for severe, refractory cases) 3

Refractory Disease or Steroid-Dependent Cases

Consider biologic therapies when conventional immunosuppressants fail or to minimize corticosteroid toxicity 5, 3:

• Biologics showed initial good response in 63% of patients in a UK cohort, with 10 of 12 patients remaining on treatment 5
• Specific biologic agents are not definitively recommended in guidelines, but TNF inhibitors and rituximab have been reported in the literature 4
• Early introduction of disease-modifying agents and biologics should be considered to minimize long-term corticosteroid adverse effects and prevent irreversible organ damage 5

Critical Management Considerations

Airway Collapse and Emergency Management

For patients with respiratory collapse requiring airway support 5:

• CPAP may be necessary to maintain airway patency in acute settings (11 of 68 patients in one series required this intervention) 5
• Airway stenting may be required for tracheobronchomalacia when medical management fails 2
• Early aggressive immunosuppressive treatment may delay or prevent irreversible cartilaginous destruction and airway collapse, making prompt recognition and treatment initiation critical 2

Disease Course and Monitoring

The disease demonstrates a relapsing course in 86% of patients, with continuous symptoms in only 14% 1:

• Anticipate unpredictable relapses interspersed with periods of remission that can be prolonged 3
• Monitor for diagnostic delay, which averages 55 weeks from symptom onset 5
• Screen for associated conditions, particularly myelodysplasia/VEXAS syndrome in men over 50 years, which requires specific treatment strategies and worsens prognosis 3

Prognostic Factors

Poor prognostic indicators include 5, 3:

• Laryngotracheal stricture
• Respiratory tract cartilage involvement
• Cardiovascular involvement
• Association with myelodysplasia/VEXAS
• Elevated serum creatinine
• Ethnicity (specific patterns noted in multivariate analysis)

Mortality occurs in approximately 18% of patients, with respiratory complications accounting for the majority of deaths 5

Common Pitfalls to Avoid

• Do not mistake early respiratory involvement for asthma—reversible airway obstruction in middle-aged adults with no prior asthma history should prompt consideration of RP 2
• Do not delay immunosuppression in respiratory disease—cartilaginous destruction becomes irreversible, and early aggressive treatment may prevent progression to airway collapse 2, 5
• Do not rely on laboratory tests for diagnosis—no specific test exists; diagnosis is clinical based on chondritis patterns and exclusion of mimics 3, 6
• Do not underestimate the diagnostic delay—maintain high clinical suspicion as formal diagnosis cannot be established before chondritis onset, which may occur years after initial symptoms 3