Lighting the fires within: the cell biology of autoinflammatory diseases

Nat Rev Immunol. 2012 Jul 25;12(8):570-80. doi: 10.1038/nri3261.

Abstract

Autoinflammatory diseases are characterized by seemingly unprovoked pathological activation of the innate immune system in the absence of autoantibodies or autoreactive T cells. Discovery of the causative mutations underlying several monogenic autoinflammatory diseases has identified key regulators of innate immune responses. Recent studies have highlighted the role of misfolding, oligomerization and abnormal trafficking of pathogenic mutant proteins in triggering autoinflammation, and suggest that more common rheumatic diseases may have an autoinflammatory component. This coincides with recent discoveries of new links between endoplasmic reticulum stress and inflammatory signalling pathways, which support the emerging view that autoinflammatory diseases may be due to pathological dysregulation of stress-sensing pathways that normally function in host defence.

Publication types

  • Research Support, N.I.H., Intramural
  • Review

MeSH terms

  • Animals
  • Autoimmune Diseases / genetics
  • Autoimmune Diseases / immunology*
  • Endoplasmic Reticulum Stress / genetics
  • Endoplasmic Reticulum Stress / immunology
  • Humans
  • Immunity, Innate / genetics
  • Immunity, Innate / immunology
  • Inflammation / genetics
  • Inflammation / immunology
  • Rheumatic Diseases / genetics
  • Rheumatic Diseases / immunology*