Recurrent hereditary fever in the era of molecular biology

Clinical and genetic aspects of recessive (Familial Mediterranean Fever - F MF - and Hyperimmunoglobulinemia IgD and Periodic Fever Syndrome - HIDS) an d dominant (Muckle-Wells Syndrome, Familial Cold Urticaria, TRAPS - TNF rec eptor associated periodic syndromes - and Cyclic Neutropenia) recurrent fev ers are reviewed. Emphasis is given to the recently identified genes respon sible for FMF, HIDS and TRAPS, MEFV, MVK and TNFR1, respectively, and to th e hypothesis concerning the function of their products.