[Hereditary intermittent fevers, other than familial Mediterranean fevers]

Abstract

Familial Mediterranean fever is no more the sole hereditary disease characterized by recurrent inflammatory attacks. Three other main entities have now been defined, both at clinical and genetic levels: a dominant disease due to mutations of one of the tumour necrosis factor receptor, called TRAPS for tumour necrosis factor receptor associated periodic syndrome, the hyper-immuno-globulinaemia D and periodic fever syndrome (HIDS), which is a metabolic disorder and the Muckle-Wells syndrome. A thorough diagnosis of these diseases is crucial for appropriate management and treatment.