Diagnostic Challenges and Management of Relapsing Polychondritis with Large-Airway Involvement: A Case Series and Literature Review
Objectives: Our aim was to investigate the diagnostic challenges and management of relapsing polychondritis (RP) with airway involvement, highlighting the need for accurate diagnosis and effective intervention to prevent severe complications. Methods: In this retrospective study, medical records fro...
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2024-09-01
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| author | I-Chun Kuo Chen-I Hsieh Yi-Chan Lee Li-Jen Hsin Wan-Ni Lin Michael J. Rutter |
| author_facet | I-Chun Kuo Chen-I Hsieh Yi-Chan Lee Li-Jen Hsin Wan-Ni Lin Michael J. Rutter |
| author_sort | I-Chun Kuo |
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| description | Objectives: Our aim was to investigate the diagnostic challenges and management of relapsing polychondritis (RP) with airway involvement, highlighting the need for accurate diagnosis and effective intervention to prevent severe complications. Methods: In this retrospective study, medical records from January 2011 through June 2024 at a single tertiary-care institution were reviewed. This study was approved by the institutional review board. A total of 34 patients were diagnosed with RP, among whom 4 presented with significant airway complications. This study focused on these four patients, detailing their clinical presentations, diagnostic processes, and outcomes following various interventions. Results: All patients were initially misdiagnosed with asthma and later developed severe airway issues necessitating interventions such as tracheotomy and endotracheal intubation. Diagnostic imaging, microlaryngoscopy and bronchoscopy (MLB) were crucial for identifying subglottic stenosis and other airway alterations. Treatments included high-dose steroids, rituximab, and surgical interventions such as balloon dilation and tracheostomy. Only one patient could be decannulated; the other three remained dependent on tracheostomy and experienced significant complications due to emergency medical interventions. Conclusions: RP can manifest with nonspecific respiratory symptoms similar to asthma, which may delay correct diagnosis and appropriate treatment, leading to critical airway complications. The early, precise identification of RP, particularly with airway involvement, is vital. MLB and dynamic expiratory CT scans play significant roles in clinical diagnosis and management. A multidisciplinary approach involving otolaryngologists, rheumatologists, and pulmonologists is essential for optimizing patient outcomes and minimizing complications. |
| format | Article |
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| institution | OA Journals |
| issn | 2075-1729 |
| language | English |
| publishDate | 2024-09-01 |
| publisher | MDPI AG |
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| spelling | doaj-art-dca099f8f21b47ab82c20f3472c01ba62025-08-20T01:55:38ZengMDPI AGLife2075-17292024-09-01149119410.3390/life14091194Diagnostic Challenges and Management of Relapsing Polychondritis with Large-Airway Involvement: A Case Series and Literature ReviewI-Chun Kuo0Chen-I Hsieh1Yi-Chan Lee2Li-Jen Hsin3Wan-Ni Lin4Michael J. Rutter5Department of Otolaryngology-Head & Neck Surgery, Chang Gung Memorial Hospital, Taoyuan 333, TaiwanDivision of Rheumatology, Allergy and Immunology, Chang Gung Memorial Hospital, Taoyuan 333, TaiwanCollege of Medicine, Chang Gung University, Taoyuan 333, TaiwanDepartment of Otolaryngology-Head & Neck Surgery, Chang Gung Memorial Hospital, Taoyuan 333, TaiwanDepartment of Otolaryngology-Head & Neck Surgery, Chang Gung Memorial Hospital, Taoyuan 333, TaiwanDivision of Pediatric Otolaryngology, Aerodigestive and Esophageal Center, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH 45229, USAObjectives: Our aim was to investigate the diagnostic challenges and management of relapsing polychondritis (RP) with airway involvement, highlighting the need for accurate diagnosis and effective intervention to prevent severe complications. Methods: In this retrospective study, medical records from January 2011 through June 2024 at a single tertiary-care institution were reviewed. This study was approved by the institutional review board. A total of 34 patients were diagnosed with RP, among whom 4 presented with significant airway complications. This study focused on these four patients, detailing their clinical presentations, diagnostic processes, and outcomes following various interventions. Results: All patients were initially misdiagnosed with asthma and later developed severe airway issues necessitating interventions such as tracheotomy and endotracheal intubation. Diagnostic imaging, microlaryngoscopy and bronchoscopy (MLB) were crucial for identifying subglottic stenosis and other airway alterations. Treatments included high-dose steroids, rituximab, and surgical interventions such as balloon dilation and tracheostomy. Only one patient could be decannulated; the other three remained dependent on tracheostomy and experienced significant complications due to emergency medical interventions. Conclusions: RP can manifest with nonspecific respiratory symptoms similar to asthma, which may delay correct diagnosis and appropriate treatment, leading to critical airway complications. The early, precise identification of RP, particularly with airway involvement, is vital. MLB and dynamic expiratory CT scans play significant roles in clinical diagnosis and management. A multidisciplinary approach involving otolaryngologists, rheumatologists, and pulmonologists is essential for optimizing patient outcomes and minimizing complications.https://www.mdpi.com/2075-1729/14/9/1194relapsing polychondritisairwaysubglottic stenosismicrolaryngobronchoscopytracheostomy |
| spellingShingle | I-Chun Kuo Chen-I Hsieh Yi-Chan Lee Li-Jen Hsin Wan-Ni Lin Michael J. Rutter Diagnostic Challenges and Management of Relapsing Polychondritis with Large-Airway Involvement: A Case Series and Literature Review Life relapsing polychondritis airway subglottic stenosis microlaryngobronchoscopy tracheostomy |
| title | Diagnostic Challenges and Management of Relapsing Polychondritis with Large-Airway Involvement: A Case Series and Literature Review |
| title_full | Diagnostic Challenges and Management of Relapsing Polychondritis with Large-Airway Involvement: A Case Series and Literature Review |
| title_fullStr | Diagnostic Challenges and Management of Relapsing Polychondritis with Large-Airway Involvement: A Case Series and Literature Review |
| title_full_unstemmed | Diagnostic Challenges and Management of Relapsing Polychondritis with Large-Airway Involvement: A Case Series and Literature Review |
| title_short | Diagnostic Challenges and Management of Relapsing Polychondritis with Large-Airway Involvement: A Case Series and Literature Review |
| title_sort | diagnostic challenges and management of relapsing polychondritis with large airway involvement a case series and literature review |
| topic | relapsing polychondritis airway subglottic stenosis microlaryngobronchoscopy tracheostomy |
| url | https://www.mdpi.com/2075-1729/14/9/1194 |
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