ABSTRACT
Fevers in children are mainly due to infection, malignancy or inflammatory conditions. Rheumatologists have an important role in the care of inflammatory conditions, many of which are associated with fevers. Seven conditions, the hereditary recurrent fever syndromes, have been defined with the presenting symptom of recurring fever, and for which mutation of a single gene has been defined: Chronic infantile neurological articular syndrome (CINCA), Familial cold autoinflammatory syndrome (FACS), Familial Mediterranean fever (FMF), hyperimmunoglobulinemia D (HIDS), Muckle-Wells syndrome (MWS), Pyogenic sterile arthritis and Pyoderma gangrenosum (PAPA) and Tumour necrosis factor receptorassociated periodic syndrome (TRAPS) . These conditions will be discussed in detail in regard to how they fit into the wider picture of pediatric rheumatological conditions, how the diagnoses may be established and the current recommended treatments for each condition.