Periodic and Non-Periodic Fevers

Abstract

Recurrent fevers are characterized by inflammatory flares separated by intervals of general overall well-being. Some conditions are caused by a genetic defect and are collectively referred to as hereditary recurrent fevers (HRF). Familial Mediterranean fever (FMF) is caused by mutations of MEFV; mevalonate kinase deficiency (MKD), by mutations of the mevalonate kinase gene (MVK); tumor necrosis factor (TNF) receptor-associated periodic fever syndrome (TRAPS), by mutations of type I TNF receptor (TNFSRF1A); cryopyrin-associated periodic syndromes (CAPS), by mutations of NLRP3. More common forms of recurrent fever syndromes include PFAPA (periodic fever, aphthosis, pharyngitis, and adenitis) syndrome, which is a multifactorial disorder.