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Case Reports
. 2019 Apr 3;12(4):e227458.
doi: 10.1136/bcr-2018-227458.

Chronic non-granulomatous supraglottitis of a male adolescent and its successful management with azathioprine

Aaron Kovacs  1 , Shankar Haran  1 , Paul Paddle  1
Affiliations
Case Reports

Chronic non-granulomatous supraglottitis of a male adolescent and its successful management with azathioprine

Aaron Kovacs et al. BMJ Case Rep. .

Abstract

Chronic non-granulomatous supraglottitis (CNGS) is a rare disorder of the supraglottic larynx, characterised by chronic supraglottic inflammation in the absence of granulomata, vasculitis, neoplasia, autoimmune disease or infective changes on histology. We present the case of a male adolescentwho attended with progressively worsening exertional dyspnoea, stridor and symptoms of obstructive sleep apnoea. Flexible nasendoscopy revealed marked supraglottic subepithelial thickening sparing the glottis and subglottis, confirmed on microlaryngoscopy. MRI of the head and neck demonstrated diffuse, homogenous supraglottic oedema. At the peak of his symptomology, the patient was admitted for further investigations and intravenous steroid therapy, and switched to prolonged oral steroids on discharge. Tracheostomy was avoided. After 3 months, he was successfully weaned from steroids to azathioprine with gradual symptomatic improvement. This case represents the first successful use of a steroid-sparing agent in the management of CNGS.

Keywords: ear, nose and throat; otolaryngology / ENT.

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Conflict of interest statement

Competing interests: None declared.

Figures

Figure 1
Figure 1
T2 MRI displaying gross supraglottic oedema.
Figure 2
Figure 2
Direct laryngoscopy demonstrating the characteristic omega-shaped epiglottis seen in epiglottitis.
Figure 3
Figure 3
Significant arytenoid inflammation evidenced on direct laryngoscopy.
Figure 4
Figure 4
Visualisation of the patient’s normal trachea.
See this image and copyright information in PMC

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