The inherited periodic fever syndromes

Inherited periodic fever syndromes are characterized by periodic fever spikes often associated with mucocutaneous and visceral symptoms. The patients affected show normal growth rate and good general conditions. Symptom-free intervals may be of variable duration. These diseases are represented by familial Mediterranean fever (FMF), hyper-IgD syndrome, tumour necrosis factor (TNF) receptor associated syndrome (TRAPS), Muckle-Wells syndrome and cold urticaria. Hereditary periodic fevers must be differentiated from recurrent upper respiratory tract infections - which are the commonest cause of recurrent fever in childhood, and from those chronic inflammatory diseases than can occasionally present with recurrent fever such as inflammatory bowel disease or Behçet's disease.