{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T23:17:27Z","timestamp":1790378247545,"version":"4.1.0"},"reference-count":28,"publisher":"MDPI AG","issue":"9","license":[{"start":{"date-parts":[[2024,9,21]],"date-time":"2024-09-21T00:00:00Z","timestamp":1726876800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Life"],"abstract":"<jats:p>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.<\/jats:p>","DOI":"10.3390\/life14091194","type":"journal-article","created":{"date-parts":[[2024,9,23]],"date-time":"2024-09-23T03:36:38Z","timestamp":1727062598000},"page":"1194","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Diagnostic Challenges and Management of Relapsing Polychondritis with Large-Airway Involvement: A Case Series and Literature Review"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1617-2507","authenticated-orcid":false,"given":"I-Chun","family":"Kuo","sequence":"first","affiliation":[{"name":"Department of Otolaryngology-Head & Neck Surgery, Chang Gung Memorial Hospital, Taoyuan 333, Taiwan"},{"name":"College of Medicine, Chang Gung University, Taoyuan 333, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3715-0363","authenticated-orcid":false,"given":"Chen-I","family":"Hsieh","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Allergy and Immunology, Chang Gung Memorial Hospital, Taoyuan 333, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yi-Chan","family":"Lee","sequence":"additional","affiliation":[{"name":"College of Medicine, Chang Gung University, Taoyuan 333, Taiwan"},{"name":"Department of Otolaryngology-Head & Neck Surgery, Chang Gung Memorial Hospital, Keelung 204, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9201-0270","authenticated-orcid":false,"given":"Li-Jen","family":"Hsin","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology-Head & Neck Surgery, Chang Gung Memorial Hospital, Taoyuan 333, Taiwan"},{"name":"College of Medicine, Chang Gung University, Taoyuan 333, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wan-Ni","family":"Lin","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology-Head & Neck Surgery, Chang Gung Memorial Hospital, Taoyuan 333, Taiwan"},{"name":"College of Medicine, Chang Gung University, Taoyuan 333, Taiwan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2257-0605","authenticated-orcid":false,"given":"Michael J.","family":"Rutter","sequence":"additional","affiliation":[{"name":"Division of Pediatric Otolaryngology, Aerodigestive and Esophageal Center, Cincinnati Children\u2019s Hospital Medical Center, Cincinnati, OH 45229, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2024,9,21]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"74","DOI":"10.7326\/0003-4819-104-1-74","article-title":"Relapsing polychondritis. Survival and predictive role of early disease manifestations","volume":"104","author":"Michet","year":"1986","journal-title":"Ann. Intern. Med."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"681","DOI":"10.1016\/S0161-6420(86)33695-9","article-title":"Ocular and systemic findings in relapsing polychondritis","volume":"93","author":"Isaak","year":"1986","journal-title":"Ophthalmology"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"193","DOI":"10.1097\/00005792-197605000-00001","article-title":"Relapsing polychondritis: Prospective study of 23 patients and a review of the literature","volume":"55","author":"McAdam","year":"1976","journal-title":"Medicine"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"114","DOI":"10.7326\/0003-4819-129-2-199807150-00011","article-title":"Relapsing polychondritis","volume":"129","author":"Trentham","year":"1998","journal-title":"Ann. Intern. Med."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"2179","DOI":"10.1007\/s10067-019-04852-3","article-title":"Diagnosing relapsing polychondritis remains a common challenge: Experience from a Chinese retrospective cohort","volume":"39","author":"Zhang","year":"2020","journal-title":"Clin. Rheumatol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"929","DOI":"10.1288\/00005537-197906000-00009","article-title":"Relapsing polychondritis\u2014Report of ten cases","volume":"89","author":"Damiani","year":"1979","journal-title":"Laryngoscope"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"347","DOI":"10.1038\/s41584-024-01113-9","article-title":"Relapsing polychondritis: Clinical updates and new differential diagnoses","volume":"20","author":"Mertz","year":"2024","journal-title":"Nat. Rev. Rheumatol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"e000788","DOI":"10.1136\/rmdopen-2018-000788","article-title":"Relapsing polychondritis: State of the art on clinical practice guidelines","volume":"4","author":"Rednic","year":"2018","journal-title":"RMD Open."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"222","DOI":"10.1111\/bjh.18220","article-title":"B-cell lymphoma mimicking relapsing polychondritis","volume":"198","author":"Dawudi","year":"2022","journal-title":"Br. J. Haematol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"96","DOI":"10.1017\/S002221512200038X","article-title":"Clinical analysis of relapsing polychondritis with airway involvement","volume":"137","author":"Zhai","year":"2023","journal-title":"J. Laryngol. Otol."},{"key":"ref_11","doi-asserted-by":"crossref","unstructured":"Handa, H., Ooka, S., Shimizu, J., Suzuki, N., and Mineshita, M. (2023). Evaluation of airway involvement and treatment in patients with relapsing polychondritis. Sci. Rep., 13.","DOI":"10.1038\/s41598-023-35616-4"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"2992","DOI":"10.1002\/art.39790","article-title":"Relapsing Polychondritis Can Be Characterized by Three Different Clinical Phenotypes: Analysis of a Recent Series of 142 Patients","volume":"68","author":"Dion","year":"2016","journal-title":"Arthritis Rheumatol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"102353","DOI":"10.1016\/j.autrev.2019.102353","article-title":"Tracheobronchial involvement of relapsing polychondritis","volume":"18","author":"Dusser","year":"2019","journal-title":"Autoimmun. Rev."},{"key":"ref_14","first-page":"2181","article-title":"Incidence and mortality of relapsing polychondritis in the UK: A population-based cohort study","volume":"54","author":"Hazra","year":"2015","journal-title":"Rheumatology"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"1024","DOI":"10.1378\/chest.08-1180","article-title":"Relapsing polychondritis and airway involvement","volume":"135","author":"Ernst","year":"2009","journal-title":"Chest"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"565","DOI":"10.1148\/radiol.2401050562","article-title":"Relapsing polychondritis: Prevalence of expiratory CT airway abnormalities","volume":"240","author":"Lee","year":"2006","journal-title":"Radiology"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1186\/s13075-022-02935-2","article-title":"Differentiating tracheobronchial involvement in granulomatosis with polyangiitis and relapsing polychondritis on chest CT: A cohort study","volume":"24","author":"Jalaber","year":"2022","journal-title":"Arthritis Res. Ther."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1177\/000348949410300410","article-title":"Proposed grading system for subglottic stenosis based on endotracheal tube sizes","volume":"103","author":"Myer","year":"1994","journal-title":"Ann. Otol. Rhinol. Laryngol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"e90","DOI":"10.1097\/RHU.0000000000001264","article-title":"Advantage of Magnetic Resonance Imaging in Detecting Tracheal Involvement and Evaluation of the Therapeutic Response in Relapsing Polychondritis With Asthma-Like Symptoms","volume":"27","author":"Sato","year":"2021","journal-title":"J. Clin. Rheumatol."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"339","DOI":"10.1007\/s00256-015-2270-x","article-title":"Imaging diagnosis in relapsing polychondritis and correlation with clinical and serological data","volume":"45","author":"Thaiss","year":"2016","journal-title":"Skeletal Radiol."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1093\/rheumatology\/kez243","article-title":"Fluorodeoxyglucose positron emission tomography\/computed tomography in the diagnosis, assessment of disease activity and therapeutic response in relapsing polychondritis","volume":"59","author":"Sharma","year":"2020","journal-title":"Rheumatology"},{"key":"ref_22","doi-asserted-by":"crossref","unstructured":"Okuda, S., Hirooka, Y., Itami, T., Nozaki, Y., Sugiyama, M., Kinoshita, K., Funauchi, M., and Matsumura, I. (2021). FDG-PET\/CT and Auricular Cartilage Biopsy Are Useful for Diagnosing with Relapsing Polychondritis in Patients without Auricular Symptoms. Life, 11.","DOI":"10.3390\/life11090956"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"282","DOI":"10.1186\/s13075-019-2083-8","article-title":"Is 18F-FDG PET\/CT useful for diagnosing relapsing polychondritis with airway involvement and monitoring response to steroid-based therapy?","volume":"21","author":"Zeng","year":"2019","journal-title":"Arthritis Res. Ther."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"897","DOI":"10.1016\/j.athoracsur.2019.02.039","article-title":"Long-Term Outcome of Metallic Stenting for Central Airway Involvement in Relapsing Polychondritis","volume":"108","author":"Wu","year":"2019","journal-title":"Ann. Thorac. Surg."},{"key":"ref_25","unstructured":"U.S. Food and Drug Administration (2024, July 21). FDA Public Health Notification: Complications from Metallic Tracheal Stents in Patients with Benign Airway Disorders, Available online: https:\/\/stening.es\/download\/articles\/FDA-complication-from-metallic-tracheal-stents.pdf."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"138","DOI":"10.1053\/j.sempedsurg.2016.02.006","article-title":"Subglottic stenosis","volume":"25","author":"Jefferson","year":"2016","journal-title":"Semin. Pediatr. Surg."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"1329","DOI":"10.1007\/s10067-013-2279-2","article-title":"Clinical characteristics and treatment outcomes of patients with relapsing polychondritis with airway involvement","volume":"32","author":"Hong","year":"2013","journal-title":"Clin. Rheumatol."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"81","DOI":"10.55563\/clinexprheumatol\/h9gq1o","article-title":"Treatment of relapsing polychondritis: A systematic review","volume":"40","author":"Petitdemange","year":"2022","journal-title":"Clin. Exp. Rheumatol."}],"container-title":["Life"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2075-1729\/14\/9\/1194\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T16:01:22Z","timestamp":1760112082000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2075-1729\/14\/9\/1194"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,9,21]]},"references-count":28,"journal-issue":{"issue":"9","published-online":{"date-parts":[[2024,9]]}},"alternative-id":["life14091194"],"URL":"https:\/\/doi.org\/10.3390\/life14091194","relation":{},"ISSN":["2075-1729"],"issn-type":[{"value":"2075-1729","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,9,21]]}}}