Chen JY, Li XY, Zong C. Relapsing polychondritis with isolated tracheobronchial involvement complicated with Sjogren's syndrome: A case report. World J Clin Cases 2022; 10(19): 6563-6570 [PMID: 35979285 DOI: 10.12998/wjcc.v10.i19.6563]
Corresponding Author of This Article
Chen Zong, MS, Chief Doctor, Department of Pulmonary and Critical Care Medicine, The Second Hospital of Shanxi Medical University, No. 382 Wuyi Road, Xinghualing District, Taiyuan 030000, Shanxi Province, China. zongchenly@126.com
Research Domain of This Article
Respiratory System
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Clin Cases. Jul 6, 2022; 10(19): 6563-6570 Published online Jul 6, 2022. doi: 10.12998/wjcc.v10.i19.6563
Relapsing polychondritis with isolated tracheobronchial involvement complicated with Sjogren's syndrome: A case report
Jun-Yan Chen, Xiao-Yan Li, Chen Zong
Jun-Yan Chen, Xiao-Yan Li, Chen Zong, Department of Pulmonary and Critical Care Medicine, The Second Hospital of Shanxi Medical University, Taiyuan 030000, Shanxi Province, China
Author contributions: Chen JY and Li XY wrote the first draft of the manuscript; Zong C wrote additional sections of the manuscript; all authors contributed to manuscript revision, read, and approved the submitted version.
Informed consent statement: The patient's consent and informed consent were obtained for this case report.
Conflict-of-interest statement: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Chen Zong, MS, Chief Doctor, Department of Pulmonary and Critical Care Medicine, The Second Hospital of Shanxi Medical University, No. 382 Wuyi Road, Xinghualing District, Taiyuan 030000, Shanxi Province, China. zongchenly@126.com
Received: November 3, 2021 Peer-review started: November 3, 2021 First decision: March 7, 2022 Revised: March 17, 2022 Accepted: May 12, 2022 Article in press: May 12, 2022 Published online: July 6, 2022 Processing time: 233 Days and 5.8 Hours
Abstract
BACKGROUND
Relapsing polychondritis (RP) is a rare, long-term, and potentially life-threatening disease characterised by recurrent paroxysmal inflammation that can involve and destroy the cartilage of the external ear, nose, larynx, and trachea.
CASE SUMMARY
We here report a case of RP involving solely the tracheobronchial cartilage ring (and not the auricular. nasal or articular cartilage) complicated by Sjögren's syndrome in a 47-year-old female whose delayed diagnosis caused a sharp decline in pulmonary function. After corticosteroid treatment, her pulmonary function improved.
CONCLUSION
In such cases, our experience suggested that 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) and fiberoptic bronchoscopy should be used to diagnose airway chondritis as relapsing polychondritis in the early phase of disease.
Core Tip: Relapsing polychondritis is a rare immune-mediated systemic disease characterized by recurrent inflammation of cartilage and proteoglycan rich tissues. In approximately 10% of patients with tracheobronchial cartilage ring involvement, characterized by cough and shortness of breath, regular bronchodilator treatment has very little effect. Deterioration of lung function occurs soon thereafter, with pulmonary function tests showing obstructive ventilation dysfunction. In this patient, the bronchial cartilage rings were undistinguishable on fiberoptic bronchoscopy, and active paratracheal inflammation revealed by positron emission tomography/computed tomography scans helped confirm the diagnosis.
