Ear
Inflammatory / infectious / autoimmune / systemic disorders
Granulomatosis with polyangiitis
Author: Nat Pernick, M.D.
Last author update: 1 October 2013
Last staff update: 14 December 2020
Copyright: 2002-2023, PathologyOutlines.com, Inc.
PubMed Search: Granulomatosis with polyangiitis [title] ear
Table of Contents
Definition / general | Terminology | Laboratory | Treatment | Microscopic (histologic) description | Differential diagnosis | Additional referencesCite this page: Pernick N. Granulomatosis with polyangiitis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/earwegenersgran.html. Accessed June 3rd, 2023.
Definition / general
- Systemic necrotizing vasculitis that typically involves kidneys, lung, upper aerodigestive tract
- Otologic involvement (otitis media, tympanic membrane perforation, sensorineural hearing loss, perforation of ear lobes, external otitis) as well as facial palsy occurs in 20 - 60% who have disease at traditional sites
Terminology
- Name changed in 2011 (J Am Soc Nephrol 2011;22:587)
Laboratory
- Elevated serum ANCA
Treatment
- Corticosteroids, immunosuppressive drugs may cause long term remission and reverse hearing loss and facial palsy
Microscopic (histologic) description
- Vasculitis, necrosis or granulomatous inflammation (few cases have all 3)
- Extra levels / recuts increases sensitivity by 7% (Ann Pathol 2005;25:87)
Differential diagnosis
- Polyarteritis nodosa: necrotizing vasculitis of small to medium sized muscular arteries
- Rheumatoid arthritis: conductive hearing loss due to involvement of incus-maleus and incus-stapes articulations
