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Review
. 2025 Jan 24;17(1):461-475.
doi: 10.21037/jtd-24-1603. Epub 2025 Jan 20.

Relapsing polychondritis: tracheobronchial involvement and differential diagnoses

Louis Grandière  1 , Hugues Vicaire  1 , Gabriel Pop  2 , Morgane Didier  1 , Olivia Freynet  1 , Marina Alexandre  3 , Dominique Clero  4 , Alexis Mathian  5   6 , Emmanuel Martinod  7   8 , Pierre-Yves Brillet  9 , Thomas Gille  8   10 , Yurdagül Uzunhan  1   8
Affiliations
Review

Relapsing polychondritis: tracheobronchial involvement and differential diagnoses

Louis Grandière et al. J Thorac Dis. .

Abstract

This review is describing the diagnostic and therapeutic approach to tracheobronchial involvement in relapsing polychondritis (RP), with a focus on differential diagnoses of inflammatory origin. RP is a systemic auto-immune disease that mainly affects cartilage structures, progressing through inflammatory flare-ups between phases of remission and ultimately leading to deformation of the involved cartilages. Besides the damage of auricular or nasal cartilage, tracheobronchial and cardiac involvement are the most severe, and can seriously alter the prognosis. Tracheobronchial lesions are assessed through a multimodal approach. Mapping of tracheal lesions is achieved using dynamic thoracic imaging and flexible bronchoscopy. Measurement of pulmonary function (with new emphasis on pulse oscillometry) is useful to diagnose obstructive ventilatory impairment, and can be used to follow RP patients, after therapeutics implementation. Diagnosis can be difficult in the absence of specific diagnostic tools, especially because there is a large number of differential diagnoses, in particular inflammatory diseases. Nuclear imaging can help with detection of metabolic activity on involved cartilages, leading to sharpen the final diagnosis. The prognosis has improved, thanks to the upgraded interventional bronchoscopy techniques, and the development of immunosuppressant including targeted therapies, such as tumor necrosis factor-α (TNF-α) inhibitors, offering patients several treatment options, in addition to supportive care.

Keywords: Relapsing polychondritis (RP); subglottic stenosis; trachea; tracheal stenosis; tracheomalacia.

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Conflict of interest statement

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://jtd.amegroups.com/article/view/10.21037/jtd-24-1603/coif). A.M. received a research grant from Sobi, participated in systemic lupus advisory board for AstraZeneca; received compensation for expert testimony for GSK; received compensation for attending meetings and/or travel from AstraZeneca, GSK, Novartis, and Otsuka; and received consultant and speaker fees from AstraZeneca, GSK, Novartis, and Otsuka. T.G. received compensation from Roche SAS and Oxyvie. Y.U. received compensation for consultancy, lecturing and conference attendance from Boehringer Ingelheim, Sanofi, CSL-Vifor, GSK, and Oxyvie. The other authors have no conflicts of interest to declare.

Figures

Figure 1
Figure 1
Thoracic CT scan, transverse section, non-injected, mediastinal window. Anterior tracheal wall thickening (arrow). CT, computed tomography.
Figure 2
Figure 2
Thoracic CT scan, transverse section, non-injected, mediastinal window. (A) Circumferential thickening of tracheal wall, responsible for tracheal stenosis (arrow). (B) Circumferential thickening of main left bronchus wall, responsible for bronchial stenosis (arrow). CT, computed tomography.
Figure 3
Figure 3
Thoracic CT scan, transverse section, non-injected, parenchymal window. Dynamic sections in inspiration (A) and expiration (B), showing a bronchial collapse during expiratory time, signaling a bronchomalacia. CT, computed tomography.
Figure 4
Figure 4
Tracheobronchial hypermetabolism during RP. Thoracic CT scan (A) related to 18-FDG PET scan (B) transverse section showing bronchial wall hypermetabolism (arrow). Thoracic CT scan (C) related to 18-FDG PET-CT (D) coronal section showing tracheobronchial wall hypermetabolism (arrows). RP, relapsing polychondritis; CT, computed tomography; 18-FDG, 18-fluorodesoxyglucose; PET, positron emission tomography.
Figure 5
Figure 5
Bronchoscopic views of tracheal stenosis.
See this image and copyright information in PMC

References

    1. Jaksch-Wartenhorst R. Polychondropathia. Wien Arch Inn Med 1923;6:93-100.
    1. Pearson CM, Kline HM, Newcomer VD. Relapsing polychondritis. N Engl J Med 1960;263:51-8. 10.1056/NEJM196007142630201 - DOI - PubMed
    1. Beck DB, Ferrada MA, Sikora KA, et al. Somatic Mutations in UBA1 and Severe Adult-Onset Autoinflammatory Disease. N Engl J Med 2020;383:2628-38. 10.1056/NEJMoa2026834 - DOI - PMC - PubMed
    1. Belot A, Duquesne A, Job-Deslandre C, et al. Pediatric-onset relapsing polychondritis: case series and systematic review. J Pediatr 2010;156:484-9. 10.1016/j.jpeds.2009.09.045 - DOI - PubMed
    1. Trentham DE, Le CH. Relapsing polychondritis. Ann Intern Med 1998;129:114-22. 10.7326/0003-4819-129-2-199807150-00011 - DOI - PubMed