Hereditary periodic fever syndromes

Autoinflammatory diseases are characterized by chronic recurrent generalized inflammation without an infectious or autoimmune etiology. The rather rare genetic autoinflammatory diseases are known as hereditary periodic fever syndromes. Over the past decade, the genetic cause of most hereditary periodic fevers has been elucidated. Familial Mediterranean Fever (FMF) due to MEFV mutations, Hyper-IgD and Periodic Fever Syndrome (HIDS) due to MVK mutations, TNF Receptor Associated Periodic Syndromes (TRAPS) due to TNFRSF1A mutations, and Cryopyrin Associated Periodic Syndromes (MAPS) due to CIAS1 mutations. The pheno…