Relapsing polychondritis with large airway involvement
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...
| Main Authors: | , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wiley
2020-01-01
|
| Series: | Respirology Case Reports |
| Subjects: | |
| Online Access: | https://doi.org/10.1002/rcr2.501 |
| _version_ | 1811286476482150400 |
|---|---|
| author | Christiaan Yu Simon A. Joosten |
| author_facet | Christiaan Yu Simon A. Joosten |
| author_sort | Christiaan Yu |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-04-13T03:00:35Z |
| format | Article |
| id | doaj.art-1115e306572f4f6d8acbee790cc29d34 |
| institution | Directory Open Access Journal |
| issn | 2051-3380 |
| language | English |
| last_indexed | 2024-04-13T03:00:35Z |
| publishDate | 2020-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Respirology Case Reports |
| spelling | doaj.art-1115e306572f4f6d8acbee790cc29d342022-12-22T03:05:26ZengWileyRespirology Case Reports2051-33802020-01-0181n/an/a10.1002/rcr2.501Relapsing polychondritis with large airway involvementChristiaan Yu0Simon A. Joosten1Monash Lung and Sleep Monash Health Clayton Victoria AustraliaMonash Lung and Sleep Monash Health Clayton Victoria AustraliaRelapsing 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.https://doi.org/10.1002/rcr2.501Non‐invasive ventilationrelapsing polychondritistracheomalacia |
| spellingShingle | Christiaan Yu Simon A. Joosten Relapsing polychondritis with large airway involvement Respirology Case Reports Non‐invasive ventilation relapsing polychondritis tracheomalacia |
| title | Relapsing polychondritis with large airway involvement |
| title_full | Relapsing polychondritis with large airway involvement |
| title_fullStr | Relapsing polychondritis with large airway involvement |
| title_full_unstemmed | Relapsing polychondritis with large airway involvement |
| title_short | Relapsing polychondritis with large airway involvement |
| title_sort | relapsing polychondritis with large airway involvement |
| topic | Non‐invasive ventilation relapsing polychondritis tracheomalacia |
| url | https://doi.org/10.1002/rcr2.501 |
| work_keys_str_mv | AT christiaanyu relapsingpolychondritiswithlargeairwayinvolvement AT simonajoosten relapsingpolychondritiswithlargeairwayinvolvement |