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

Maughan, EF; Michaels, J; Miller, B; Weir, J; Salama, A; Warner, E; Ghufoor, K; ... Al Yaghchi, C; + view all (2020) Primary Immunoglobulin G4-Related Laryngeal Disease: A Case Series and Review of Literature. Clinical Medicine Insights: Case Reports , 13 pp. 1-6. 10.1177/1179547620960197. Green open access

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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.

Type: Article
Title: Primary Immunoglobulin G4-Related Laryngeal Disease: A Case Series and Review of Literature
Open access status: An open access version is available from UCL Discovery
DOI: 10.1177/1179547620960197
Publisher version: https://doi.org/10.1177%2F1179547620960197
Language: English
Additional information: © 2021 by SAGE Publications Ltd. This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License (https://creativecommons.org/licenses/by-nc/4.0/).
Keywords: Laryngeal fibrosis, IgG4-RD, IgG4-related disease
UCL classification: UCL
UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Renal Medicine
URI: https://discovery.ucl.ac.uk/id/eprint/10125144
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