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Padoan et al. Vessel Plus 2021;5:41 Vessel Plus
DOI: 10.20517/2574-1209.2021.41

Review Open Access

Ear, nose, and throat in ANCA-associated vasculitis:

a comprehensive review

2,3
1
1
1
Roberto Padoan , Debora Campaniello , Mara Felicetti , Diego Cazzador , Franco Schiavon 1
1
Rheumatology Unit, Department of Medicine DIMED, University of Padova, Padova 35128, Italy.
2
Otorhinolaryngology Unit, Department of Neurosciences, University of Padova, Padova 35128, Italy.
3
Section of Human Anatomy, Department of Neurosciences, University of Padova, Padova 35128, Italy.
Correspondence to: Dr. Roberto Padoan, Rheumatology Unit, University of Padova, Via Giustiniani 2, Padova 35128, Italy.
E-mail: robertopadoan.reumatologia@gmail.com
How to cite this article: Padoan R, Campaniello D, Felicetti M, Cazzador D, Schiavon F. Ear, nose, and throat in ANCA-associated
vasculitis: a comprehensive review. Vessel Plus 2021;5:41. https://dx.doi.org/10.20517/2574-1209.2021.41
Received: 28 Feb 2021 First Decision: 23 Apr 2021 Revised: 1 May 2021 Accepted: 21 May 2021 First online: 24 May 2021

Academic Editor: Luca Quartuccio Copy Editor: Yue-Yue Zhang Production Editor: Yue-Yue Zhang

Abstract
Ear, nose, and throat (ENT) involvement is a common feature in antineutrophil cytoplasmic antibody (ANCA)-
associated vasculitis (AAV), particularly in granulomatosis with polyangiitis and eosinophilic granulomatosis with
polyangiitis. Over the last decade, substantial advancement has been made in understanding AAV pathogenesis,
classification, and treatment. Typical ENT symptoms may include sinonasal, otologic, pharyngeal, and laryngeal
manifestations. The otolaryngologic symptoms of AAV sometimes might be misdiagnosed in etiology as infectious
or allergic. Thus, rapid recognition and early diagnosis of AAV as the cause of the symptoms prevent the risk of
irreversible organ damage. The high impact of ENT symptoms on the quality of life of AAV patients confirms the
importance of their early treatment through specific local and systemic approaches. Appropriate interdisciplinary
management to early recognition of AAV and initiation of treatment may reduce morbidity in these patients. The
purpose of this comprehensive review is to describe the clinical, histological, and radiological findings of ENT
involvement in AAV and to update their surgical and therapeutic management, with a focus also on the role of a
multidisciplinary team, involving the otorhinolaryngologist.

Keywords: Antineutrophil cytoplasmic antibody, ANCA-associated vasculitis, granulomatosis with polyangiitis,
microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis, ear, nose, throat

© The Author(s) 2021. Open Access This article is licensed under a Creative Commons Attribution 4.0
International License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, sharing,
adaptation, distribution and reproduction in any medium or format, for any purpose, even commercially, as
long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and
indicate if changes were made.

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