Updates on autoinflammatory diseases

Published by Elsevier Ltd..

Autoinflammatory diseases are hyperinflammatory, immune dysregulatory diseases caused by innate immune cells dysregulation that present typically in the perinatal period with systemic and organ-targeted inflammation, but with improved genetic testing and the development of diagnostic criteria, milder and later-onset forms are being detected in adulthood. While the discovery of gain-of-function mutations in innate sensors linked to the production of proinflammatory cytokines provided the bases for anti-cytokine therapies that changed disease and patient outcomes, the field is expanding with the increasing discovery of disease-causing loss-of-function mutations in genes with cellular house-keeping functions that affect cell homeostasis and when dysregulated trigger innate inflammatory pathways. This review focuses on updates on molecular pathways and diseases that cause predominantly IL-1β and Type-I IFN-mediated autoinflammatory diseases.

Medienart:

E-Artikel

Erscheinungsjahr:

2018

Erschienen:

2018

Enthalten in:

Zur Gesamtaufnahme - volume:55

Enthalten in:

Current opinion in immunology - 55(2018) vom: 05. Dez., Seite 97-105

Sprache:

Englisch

Beteiligte Personen:

Martinez-Quiles, Narcisa [VerfasserIn]
Goldbach-Mansky, Raphaela [VerfasserIn]

Links:

Volltext

Themen:

Interferon Type I
Journal Article
Research Support, N.I.H., Intramural
Research Support, Non-U.S. Gov't
Review

Anmerkungen:

Date Completed 25.09.2019

Date Revised 08.04.2022

published: Print-Electronic

Citation Status MEDLINE

doi:

10.1016/j.coi.2018.09.014

funding:

Förderinstitution / Projekttitel:

PPN (Katalog-ID):

NLM29085248X