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[Laryngotracheobronchial involvement in relapsing polychondritis]
1Department of Otorhinolaryngology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing 100730.
Summary
Relapsing polychondritis (RP) can severely affect the airway, leading to respiratory complications. Despite treatments, airway involvement remains a major cause of mortality in RP patients.
Area of Science:
- Rheumatology
- Pulmonology
- Otolaryngology
Context:
- Relapsing polychondritis (RP) is a rare, systemic autoimmune disease characterized by progressive cartilage destruction.
- Laryngotracheobronchial involvement is a serious complication of RP, impacting patient morbidity and mortality.
Purpose:
- To analyze the clinical presentation, therapeutic interventions, and outcomes of laryngotracheobronchial involvement in patients with relapsing polychondritis.
- To identify risk factors and prognostic indicators for respiratory complications in RP.
Summary:
- A retrospective review of 13 RP patients with laryngotracheobronchial involvement revealed high rates of laryngeal, tracheal, and bronchial lesions (46%, 69%, 69% respectively).
- Treatments included corticosteroids, immunosuppressants, dapsone, tracheotomy, and stents, with a 5-year survival rate of 78.6%.
- Two deaths occurred due to respiratory complications, highlighting the severity of airway involvement.
Impact:
- Respiratory tract compromise is the most critical manifestation of relapsing polychondritis.
- Effective management strategies are crucial to improve survival rates in RP patients with airway involvement.