Related Experiment Video
Updated: Jun 26, 2025
04:44
Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
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[Tracheobronchial involvement in relapsing polychondritis and differential diagnoses]
L Grandière1, T Gille2, P-Y Brillet3
1Service de pneumologie, centre de référence constitutif des maladies pulmonaires rares, hôpital Avicenne, Assistance publique-Hôpitaux de Paris - Hôpitaux universitaires de Paris Seine-Saint-Denis, Bobigny, France.
Revue Des Maladies Respiratoires
|May 18, 2024
Summary
Relapsing polychondritis is a systemic autoimmune disease causing cartilage inflammation and deformation. Early diagnosis and advanced treatments like interventional bronchoscopy and targeted therapies improve patient prognosis.
Area of Science:
- Rheumatology and Immunology
- Pulmonology
- Cardiology
Context:
- Relapsing polychondritis (RP) is a rare systemic autoimmune disease targeting cartilage.
- It involves progressive inflammation and deformation of cartilaginous structures, including ears, nose, trachea, and heart.
- Severe tracheobronchial and cardiac involvement significantly impacts patient prognosis.
Purpose:
- To outline the diagnostic challenges and current management strategies for relapsing polychondritis.
- To highlight the importance of multimodal assessment for tracheobronchial lesions.
- To discuss the improving prognosis due to advancements in treatment.
Summary:
- RP presents with inflammatory flares and remissions, leading to cartilage damage.
- Diagnostic difficulties arise from the lack of specific tools and numerous differential diagnoses.
- Tracheobronchial involvement necessitates dynamic thoracic imaging, pulmonary function tests (e.g., pulse oscillometry), and flexible bronchoscopy for lesion mapping.
Impact:
- Improved diagnostic accuracy through multimodal approaches enhances patient outcomes.
- Advancements in interventional bronchoscopy offer effective management for airway compromise.
- Development of immunosuppressants and targeted therapies provides new treatment avenues, improving the overall prognosis for RP patients.
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