[The "self-inflammatory syndrome"]

The "self-inflammatory syndrome" gathers diseases all characterized by a recurrent inflammatory syndrome with fever, in the absence of infection or neoplasia. It is based on a genetic support characterized by mutations in genes implied in the inflammatory response and in the activation of the cytokine network. The diseases associated with this syndrome are familial Mediterranean fever (FMF), TRAPS (tumor necrosis factor receptor super family 1 A-associated periodic syndrome), familial cold urticaria, the Muckle-Wells syndrome, the hyper IgD syndrome and CINCA. The clinical symptoms of all these diseases include in the auto-inflammatory syndrome are quite similar: recurrent attacks, with fever, articular, abdominal, cutaneous symptoms, and an inflammatory syndrome.