title: Periodic fevers and autoinflammatory syndromes in childhood.
abstract: Recurrent fever is a common presentation in paediatric practice and can be caused by a wide variety of diseases including autoinflammatory conditions. The innate immune system plays an essential role in the 'first line' response to infection through mediation of inflammatory responses. Inflammasomes are part of the regulatory process for this system and result in the production of the powerful pro-inflammatory cytokine interleukin-1B. Dysregulation of inflammasomes, and Interleukin 1 production, contributes to the pathogenesis of autoinflammatory diseases. This review focuses on described periodic fever syndromes (PFS) which are now collectively referred to as autoinflammatory syndromes. Conditions discussed include periodic fever aphthous stomatitis pharyngitis and cervical adenopathy, familial Mediterranean fever, tumour necrosis factor receptor-associated periodic syndromes, hyperimmunoglobulinaemia D and the cryopyrin-associated periodic syndromes. Presenting features, complications, diagnostic and treatment approaches for these conditions are discussed. Nonetheless, as most of these conditions are rare and may have significant long-term complications, it is recommended that they be managed in consultations with a physician experienced in managing PFS.
authors.affiliation: Rheumatology Department, Starship Children's Hospital, Auckland, New Zealand. gostring@adhb.govt.nz.|Paediatrics Child and Youth Health, University of Auckland, Auckland, New Zealand. gostring@adhb.govt.nz.
authors.forename: Genevieve T
authors.initials: GT
authors.lastname: Ostring
authors.affiliation: General Medicine, The Sydney Children's Hospitals Network, New South Wales, Australia.|Department of Paediatrics and Child Health, University of Sydney, Sydney, New South Wales, Australia.|Department of Child and Maternal Health, University of New South Wales, Sydney, New South Wales, Australia.
authors.forename: Davinder
authors.initials: D
authors.lastname: Singh-Grewal
mesh_terms: D002648:Child; D003937:Diagnosis, Differential; D056660:Hereditary Autoinflammatory Diseases / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D006801:Humans
keywords: general paediatrics; rheumatology