Autoinflammation

WILDNER Gerhild

Purpose

The primary reaction of the immune system to dangerous events for the body is inflammation, mediated by cells of the innate immune system acting as sentinels for the integrity of the body. Those cells have receptors recognizing general pattern of invading pathogens or sick or dying cells of the body. The inflammation induced in response to activation of these pattern recognition receptors (e. g. Toll-like receptors or inflammasomes) aims at fighting and killing pathogens. Since this recognition is not very specific, such pattern can be accidentally provided by the own body, recognized by innate immune cells, confused with dangerous events and thus initiating inflammation, leading to autoinflammation.

Methods

Autoinflammation describes a disturbed activation of the innate immune system, activated by local-tissue-specific factors (e. g. urate crystals in gout) with a predominant role for IL-1 and the inflammasome. T and B cells do not play a role, unless they get activated by secondary events leading to autoantigen recognition.

Results

Autoinflammatory syndromes are hereditary, typically monogenic diseases affecting components of the inflammasome and leading to a dysregulation of the cytokine balance, followed by systemic inflammation mediated by macrophages and granulocytes. Affected genes are components of inflammasomes (NLRP, NOD) or TNFR1. Fever is a cardinal symptom, often together with other symptoms of various organs including the eye like conjunctivitis and uveitis in Blau syndrome.

Conclusions

The borderline between autoinflammation and autoimmunity is not definite, some diseases like HLA-B27-associated uveitis and Behçet's syndrome display a mixed pattern of both. In my talk I will present immune mechanisms leading to autoinflammation and resulting therapeutic aspects.




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