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Relapsing polychondritis: tracheobronchial involvement and differential diagnoses
Louis Grandière1, Hugues Vicaire1, Gabriel Pop2
1Pulmonology Department, Reference Center for Rare Pulmonary Diseases, Hôpital Avicenne, Assistance Publique - Hôpitaux de Paris - Hôpitaux Universitaires de Paris Seine-Saint-Denis, Bobigny, France.
Journal of Thoracic Disease
|February 20, 2025
Summary
Relapsing polychondritis (RP) can affect the airways, requiring a multimodal diagnostic approach. Early diagnosis and targeted therapies, including TNF-α inhibitors, improve outcomes for this rare autoimmune disease.
Area of Science:
- Rheumatology
- Pulmonology
- Immunology
Background:
- Relapsing polychondritis (RP) is a rare systemic autoimmune disease targeting cartilage.
- Tracheobronchial involvement in RP is severe, significantly impacting prognosis.
- Differential diagnosis is challenging due to overlapping inflammatory conditions.
Purpose of the Study:
- To review diagnostic and therapeutic strategies for tracheobronchial involvement in RP.
- To highlight differential diagnoses of inflammatory origin.
- To discuss advancements in managing RP-related airway complications.
Main Methods:
- Multimodal assessment including dynamic thoracic imaging and flexible bronchoscopy.
- Pulmonary function tests, emphasizing pulse oscillometry for obstructive ventilatory impairment.
- Nuclear imaging for detecting metabolic activity in affected cartilages.
Main Results:
- Tracheobronchial lesions require comprehensive evaluation for accurate diagnosis.
- Pulse oscillometry aids in diagnosing and monitoring ventilatory impairment.
- Nuclear imaging assists in sharpening the diagnosis by identifying active inflammation.
Conclusions:
- Improved diagnostic tools and interventional bronchoscopy enhance management.
- Immunosuppressants, particularly TNF-α inhibitors, offer effective treatment options.
- A combination of therapies and supportive care improves the prognosis for RP patients with airway involvement.
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