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Autoinflammatory Syndromes in Children. Systemic autoinflammatory diseases are rare disorders of innate immunity which usually present in childhood with recurrent or continuous attacks of fever and systemic inflammation. The discovery of the genetic defect underlying Familial Mediterranean fever in 1997 has proved exceptionally informative about the innate immune system and the regulation of pro inflammatory cytokines particularly IL-1. Although extremely rare, systemic autoinflammatory diseases are important to recognise as many can now be completely controlled by long term drug therapies. Diagnosis relies on clinical suspicion followed by genetic testing. This review will focus on the main systemic autoinflammatory diseases.