Familial Mediterranean fever: the molecular pathways from stress exposure to attacks

Rheumatology (Oxford). 2020 Dec 1;59(12):3611-3621. doi: 10.1093/rheumatology/keaa450.

Abstract

FMF is an autoinflammatory disease characterized by recurrent attacks and increased IL-1 synthesis owing to activation of the pyrin inflammasome. Although knowledge of the mechanisms leading to the activation of pyrin inflammasome is increasing, it is still unknown why the disease is characterized by attack. The emergence of FMF attacks after emotional stress and the induction of attacks with metaraminol in previous decades suggested that stress-induced sympathoadrenal system activation might play a role in inflammasome activation and triggering attacks. In this review, we will review the possible molecular mechanism of stress mediators on the inflammation pathway and inflammasome activation. Studies on stress mediators and their impact on inflammation pathways will provide a better understanding of stress-related exacerbation mechanisms in both autoinflammatory and autoimmune diseases. This review provides a new perspective on this subject and will contribute to new studies.

Keywords: Familial Mediterranean fever; attacks; catecholamines; epinephrine; glucocorticoids; inflammasome; norepinephrine; stress.

Publication types

  • Review

MeSH terms

  • Familial Mediterranean Fever / etiology*
  • Glucocorticoids / physiology
  • Humans
  • Immune System / physiology
  • Inflammasomes / physiology
  • Signal Transduction
  • Stress, Psychological / complications*
  • Sympathoadrenal System / physiology

Substances

  • Glucocorticoids
  • Inflammasomes