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. 2022 Oct 28;24(1):241.
doi: 10.1186/s13075-022-02935-2.

Differentiating tracheobronchial involvement in granulomatosis with polyangiitis and relapsing polychondritis on chest CT: a cohort study

Carole Jalaber  1 , Xavier Puéchal  2 , Ines Saab  1 , Emma Canniff  1 , Benjamin Terrier  2   3 , Luc Mouthon  2   3 , Eglantine Cabanne  1   3 , Sandra Mghaieth  1 , Marie-Pierre Revel  1   3 , Guillaume Chassagnon  4   5
Affiliations

Differentiating tracheobronchial involvement in granulomatosis with polyangiitis and relapsing polychondritis on chest CT: a cohort study

Carole Jalaber et al. Arthritis Res Ther. .

Abstract

Background: In patients with tracheobronchial involvement, the differential diagnosis between granulomatosis with polyangiitis (GPA) and relapsing polychondritis (RP) can be challenging. The aim of this study was to describe the characteristics of airway abnormalities on chest computed tomography (CT) in patients with GPA or RP and to determine whether specific imaging criteria could be used to differentiate them.

Methods: GPA and RP patients with tracheobronchial involvement referred to a national referral center from 2008 to 2020 were evaluated. Their chest CT images were reviewed by two radiologists who were blinded to the final diagnosis in order to analyze the characteristics of airway involvement. The association between imaging features and a diagnosis of GPA rather than RP was analyzed using a generalized linear regression model.

Results: Chest CTs from 26 GPA and 19 RP patients were analyzed. Involvement of the subglottic trachea (odds ratio for GPA=28.56 [95% CI: 3.17; 847.63]; P=0.001) and extensive airway involvement (odds ratio for GPA=0.02 [95% CI: 0.00; 0.43]; P=0.008) were the two independent CT features that differentiated GPA from RP in multivariate analysis. Tracheal thickening sparing the posterior membrane was significantly associated to RP (odds ratio for GPA=0.09 [95% CI: 0.02; 0.39]; P=0.003) but only in the univariate analysis and suffered from only moderate interobserver agreement (kappa=0.55). Tracheal calcifications were also associated with RP only in the univariate analysis (odds ratio for GPA=0.21 [95% CI: 0.05; 0.78]; P=0.045).

Conclusion: The presence of subglottic involvement and diffuse airway involvement are the two most relevant criteria in differentiating between GPA and RP on chest CT. Although generally considered to be a highly suggestive sign of RP, posterior tracheal membrane sparing is a nonspecific and an overly subjective sign.

Keywords: Granulomatosis with polyangiitis; Multidetector computed tomography; Relapsing polychondritis; Respiratory tract diseases; Trachea.

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Conflict of interest statement

XP has been an investigator in academic studies of ANCA-associated vasculitis for which rituximab was provided by Roche Pharma. MPR reports personal fees from Boehringer Ingelheim, Bracco, Chiesi, and GE Healthcare; travel fees from Guerbet; and a research grant from the French Ministry of Health and the French Cancer Institute, outside the submitted work. G. C. reports personal fees from Chiesi and Gleamer outside the submitted work. None declared: C. J., I. S., Em. C., B. T., L. M., Eg. C., and S. M.

Figures

Fig. 1
Fig. 1
Flow chart (GPA granulomatosis with polyangiitis, RP relapsing polychondritis)
Fig. 2
Fig. 2
Granulomatosis with polyangiitis. A Coronal reformation of the unenhanced chest computed tomography shows focal tracheal wall thickening (arrow). B On the axial image, the tracheal wall thickening (arrow) is circumferential and responsible for lumen stenosis. C Below, the thoracic trachea is normal
Fig. 3
Fig. 3
Relapsing polychondritis. A Unenhanced chest computed tomography shows tracheal wall thickening sparing the posterior membrane and responsible for lumen stenosis (arrow). B On coronal reformation, the tracheal wall thickening is extensive (arrows) and extends to the left main bronchus (arrowhead)
Fig. 4
Fig. 4
Atypical tracheal wall thickenings. A Unenhanced chest computed tomography (CT) shows tracheal wall thickening sparing the posterior membrane (arrow) in a patient with granulomatosis with polyangiitis. B Unenhanced chest CT showing circumferential tracheal wall thickening (arrow) in a patient with relapsing polychondritis
See this image and copyright information in PMC

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