Monocytes, Macrophages, and Microglia and the Role of IL-1 in Autoimmune Inner Ear Disease (AIED)

Monocytes, Macrophages, and Microglia and the Role of IL-1 in Autoimmune Inner Ear Disease (AIED) Purpose of Review Autoimmune inner ear disease (AIED) is a rare disease of unknown etiology characterized by a sudden decline in hearing. Although AIED is initially responsive to corticosteroid therapy, this response is lost over time. We recently demonstrated in a small cohort of corticosteroid-resistant AIED patients that IL-1 was overexpressed in the plasma and that IL-1 antagonism led to hearing improvement and reduction in circulating IL-1. Autoinflammatory diseases, such as Muckle-Wells syndrome (MWS), are diseases of the innate immune system whose phenotype includes sensorineural hearing loss. Monocytes are the primary cells orches- trating the inflammatory response in this family of diseases, due to a gain-of-function mutation that causes excessive IL-1 release. Recent Findings We have observed that monocytes from AIED patients release excessive IL-1 and behave similarly to mono- cytes from Muckle-Wells patients, despite the lack of pathogenic mutations and other characteristic phenotypic manifestations. Unlike MWS, we have observed substantially greater hearing improvement with IL-1 antagonism than hearing improvement reported in MWS. Summary The role of monocytes, macrophages, and microglia and the cytokines they secrete are of critical importance in both understanding disease mechanism and identification of novel therapeutic targets to treat chronic inflammatory diseases of the cochlea http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Current Otorhinolaryngology Reports Springer Journals

Monocytes, Macrophages, and Microglia and the Role of IL-1 in Autoimmune Inner Ear Disease (AIED)

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Publisher
Springer US
Copyright
Copyright © 2018 by Springer Science+Business Media, LLC, part of Springer Nature
Subject
Medicine & Public Health; Otorhinolaryngology
eISSN
2167-583X
D.O.I.
10.1007/s40136-018-0191-7
Publisher site
See Article on Publisher Site

Abstract

Purpose of Review Autoimmune inner ear disease (AIED) is a rare disease of unknown etiology characterized by a sudden decline in hearing. Although AIED is initially responsive to corticosteroid therapy, this response is lost over time. We recently demonstrated in a small cohort of corticosteroid-resistant AIED patients that IL-1 was overexpressed in the plasma and that IL-1 antagonism led to hearing improvement and reduction in circulating IL-1. Autoinflammatory diseases, such as Muckle-Wells syndrome (MWS), are diseases of the innate immune system whose phenotype includes sensorineural hearing loss. Monocytes are the primary cells orches- trating the inflammatory response in this family of diseases, due to a gain-of-function mutation that causes excessive IL-1 release. Recent Findings We have observed that monocytes from AIED patients release excessive IL-1 and behave similarly to mono- cytes from Muckle-Wells patients, despite the lack of pathogenic mutations and other characteristic phenotypic manifestations. Unlike MWS, we have observed substantially greater hearing improvement with IL-1 antagonism than hearing improvement reported in MWS. Summary The role of monocytes, macrophages, and microglia and the cytokines they secrete are of critical importance in both understanding disease mechanism and identification of novel therapeutic targets to treat chronic inflammatory diseases of the cochlea

Journal

Current Otorhinolaryngology ReportsSpringer Journals

Published: Apr 10, 2018

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