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Case Reports
. 2020 Oct 28:13:1179547620960197.
doi: 10.1177/1179547620960197. eCollection 2020.

Primary Immunoglobulin G4-Related Laryngeal Disease: A Case Series and Review of Literature

Elizabeth F Maughan  1 , Joshua Michaels  2 , Benjamin Miller  1 , Justin Weir  3 , Alan Salama  4 , Elinor Warner  2 , Khalid Ghufoor  2 , Gurpreet Sandhu  1 , Chadwan Al Yaghchi  1
Affiliations
Case Reports

Primary Immunoglobulin G4-Related Laryngeal Disease: A Case Series and Review of Literature

Elizabeth F Maughan et al. Clin Med Insights Case Rep. .

Abstract

Objective: Immunoglobulin G4-related disease (IgG4-RD) is an increasingly recognised cause of various systemic fibro-inflammatory conditions. However, laryngeal involvement as a primary feature is extremely rare. We aimed to report on a case series of such patients and examine the global literature relating to laryngeal involvement.

Methods: Having previously reported a case of IgG4-RD laryngeal pseudotumour, we describe a case series of further 4 patients with primary laryngeal IgG4-RD managed by our UK quaternary airway service and provide a brief overview of laryngeal IgG4-RD.

Results: Including our cases, 14 cases of primary laryngeal IgG4-RD have been reported. Vocal cord involvement is relatively uncommon. Repeat biopsies may be required to achieve histological diagnosis. Remission is achievable by commencement of immunomodulatory treatment, following which laryngeal reconstruction may be necessary.

Conclusion: Laryngeal involvement is a rare presentation of IgG4-RD, itself a rare and difficult-to-diagnose condition. A high and prolonged index of suspicion is necessary from both surgical and pathological specialists for correct diagnosis and management.

Keywords: IgG4-RD; IgG4-related disease; Laryngeal fibrosis.

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Conflict of interest statement

Declaration of conflicting interests:The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Figures

Figure 1.
Figure 1.
Intraoperative images of Case 1’s supraglottic stenosis (A) pre-laryngotracheal reconstruction (LTR), (B) during LTR and (C) on removal of stent 2 weeks post-laryngotracheal reconstruction.
Figure 2.
Figure 2.
Intraoperative images of supraglottic stenosis in Case 4 (A) pre-treatment and (B) following laser ‘pepperpotting’ to release the tight supraglottic fibrosis.
Figure 3.
Figure 3.
(A) Haematoxylin & Eosin (H&E) staining of laryngeal biopsy demonstrating fibrosis with plasma cell infiltrates. Immunostaining demonstrating (B) Widespread IgG positivity and (C) IgG4 positive cells within the submucosa.
See this image and copyright information in PMC

References

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