How autoinflammation may turn into autoimmune inflammation: Insights from monogenetic and complex IL-1 mediated auto-inflammatory diseases

N. M. ter Haar, M. H.A. Jansen, J. F. Frenkel, S. J. Vastert*

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

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Abstract

IL-1 mediated auto-inflammatory diseases are characterised by episodes of unexplained fever, generalized and localized inflammation. The characteristic symptoms predominantly result from exaggerated activation of innate immune pathways. However, in some patients with typical IL-1 mediated diseases, chronic disease manifestations develop in the absence of acute inflammation, suggesting the involvement of adaptive immune pathways. We discuss clinical observations as well as novel insights in how chronic activation of innate immune pathways can lead to auto-immune disease features in patients with auto-inflammatory diseases and how we need to better understand these sequelae in order to improve treatment strategies.

Original languageEnglish
Article number108538
Number of pages8
JournalClinical Immunology
Volume219
DOIs
Publication statusPublished - Oct 2020

Keywords

  • Auto-immune inflammation
  • Auto-inflammation
  • Mechanisms of disease

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