Summary of the pathogenesis of FMF. With trigger, inflammasome formation process is triggered in Pyrin mutants (1 and 2a). Cryopyrin protein and procaspase 1 complex into an inflammasome due to nonfunctioning Pyrin (2b and 2c). Procaspase 1 turns into Caspase 1 by the inflammasome action (3). IL-1β is maturated by Caspase 1 (4). IL-1β binds to the receptor (5a). IL-1β signaling cascade followed by inflammation is activated by the binding of IL-1β (5b). Signaling by the Receptor-IL-1β complex leads to further transcription of proIL-1β, resulting in an increase in IL-1β levels. Here, we had only considered the Caspase 1 independent processing of IL-1β (5c). Signaling by the Receptor-IL-1β complex also stimulates the antagonist production (5d). Binding of antagonist to the receptor does not result in active IL-1β signaling (5e).