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Case Reports
. 2022 Jul 6;10(19):6563-6570.
doi: 10.12998/wjcc.v10.i19.6563.

Relapsing polychondritis with isolated tracheobronchial involvement complicated with Sjogren's syndrome: A case report

Jun-Yan Chen  1 , Xiao-Yan Li  1 , Chen Zong  2
Affiliations
Case Reports

Relapsing polychondritis with isolated tracheobronchial involvement complicated with Sjogren's syndrome: A case report

Jun-Yan Chen et al. World J Clin Cases. .

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.

Keywords: 18F-fluorodeoxyglucose positron emission tomography/computed tomography; Case report; Fiberoptic bronchoscopy; Relapsing polychondritis; Tracheobronchial involvement.

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Conflict of interest statement

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.

Figures

Figure 1
Figure 1
Respiratory function tests. A: December, 2018; B: May, 2020. Compared with previous measurements, the expiratory flow was impaired and exhibited a platform-like change, suggestive of variable intrathoracic (upper airway) obstruction.
Figure 2
Figure 2
Chest X-ray. The trachea is in the centre and the textures of both lungs are enhanced.
Figure 3
Figure 3
Computed tomography analysis. A and B: The diameters of the trachea and bilateral bronchi show narrowing in the expiratory phase (B) compared to the inspiratory phase (A); C and D: The axial computed tomography image of mediastinal field for inspiration (C) and expiration (D).
Figure 4
Figure 4
Bronchoscopy. The tracheal cartilage ring appears blurred and the tracheal mucosa evidences swelling. A: Tracheal juga; B: Left principal bronchus; and C: Right primary bronchus.
Figure 5
Figure 5
Fluorodeoxyglucose positron emission tomography/computed tomography imaging. A-C: The walls of the trachea and left and right main bronchi show even thickening (with obvious thickening of the tracheal cartilage). Metabolic activities in these structures are slightly to moderately enhanced, especially near the hilum. The metabolic distributions were figure-eight- or strip-shaped. SUVmax = 2.91.
Figure 6
Figure 6
Lower lip’s salivary gland tissue biopsy (magnification, 200 ×). A focal region with > 50 lymphocytes is depicted.
See this image and copyright information in PMC

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References

    1. Borgia F, Giuffrida R, Guarneri F, Cannavò SP. Relapsing Polychondritis: An Updated Review. Biomedicines. 2018;6 - PMC - PubMed
    1. Hazra N, Dregan A, Charlton J, Gulliford MC, D'Cruz DP. Incidence and mortality of relapsing polychondritis in the UK: a population-based cohort study. Rheumatology (Oxford) 2015;54:2181–2187. - PubMed
    1. de Montmollin N, Dusser D, Lorut C, Dion J, Costedoat-Chalumeau N, Mouthon L, Chassagnon G, Revel MP, Puéchal X. Tracheobronchial involvement of relapsing polychondritis. Autoimmun Rev. 2019;18:102353. - PubMed
    1. Sharma A, Gnanapandithan K, Sharma K, Sharma S. Relapsing polychondritis: a review. Clin Rheumatol. 2013;32:1575–1583. - PubMed
    1. Behar JV, Choi YW, Hartman TA, Allen NB, McAdams HP. Relapsing polychondritis affecting the lower respiratory tract. AJR Am J Roentgenol. 2002;178:173–177. - PubMed

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