Relapsing polychondritis with large airway involvement.
Christiaan YuSimon A JoostenPublished in: Respirology case reports (2019)
Relapsing polychondritis is a rare autoimmune condition characterized by episodic and progressive cartilaginous inflammation. Its clinical presentation is vastly divergent and can affect various organs. We report the uncommon case of large airway involvement in a patient presenting with shortness of breath on the background of diagnosed relapsing polychondritis. Computed tomography (CT) chest demonstrated thickening of the cartilaginous portions of the trachea and bronchi with sparing of the posterior membranes, consistent with tracheobronchomalacia and repeated cartilaginous destruction. High doses of systemic glucocorticoids, accompanied by continuous positive airway pressure, were required for treatment. We highlight the importance of identifying the extent of airways affected and definitive positive airway pressure support for relapsing polychondritis affecting major airways in addition to conventional therapy of immunosuppression.
Keyphrases
- multiple sclerosis
- positive airway pressure
- obstructive sleep apnea
- sleep apnea
- computed tomography
- cystic fibrosis
- case report
- positron emission tomography
- dual energy
- disease activity
- image quality
- oxidative stress
- magnetic resonance imaging
- contrast enhanced
- combination therapy
- locally advanced
- magnetic resonance