title: [Genetic fever syndromes. Hereditary recurrent (periodic) fever syndromes].
abstract: Genetic fever syndromes or hereditary recurrent fever syndromes (HRF) are considered to be part of the autoinflammatory diseases (AID) which result from errors in the innate immune system. Patients typically have self-limiting episodes of fever and high levels of inflammation markers. The mode of inheritance is autosomal recessive or autosomal dominant. The diseases of the HRF include familial Mediterranean fever, tumor necrosis factor receptor 1-associated periodic syndrome, hyper-IgD syndrome and cryopyrin-associated periodic fever syndromes. The disease known as deficiency of interleukin 1 (IL1) receptor antagonist does not fully belong to this group because fever is not a typical symptom. The therapy depends on the type and severity of the disease. Effective prophylaxis is possible for FMF. Biologicals, especially IL1 blocking agents are highly effective in very severe fever syndromes. In order to collect more information on AID, to establish a biobank and coordinate research in this field the AID-Net project was founded. Currently 606 patients with AID are registered of whom 381 have HRF.
authors.affiliation: Zentrum für Kinder- und Jugendmedizin, Klinik für Kinderheilkunde III - Bereiche Kardiologie und Rheumatologie, Hufelandstr. 55, 45122 Essen. ulrich.neudorf@uk-essen.de
authors.forename: U
authors.initials: U
authors.lastname: Neudorf
authors.forename: E
authors.initials: E
authors.lastname: Lainka
authors.forename: T
authors.initials: T
authors.lastname: Kallinich
authors.forename: D
authors.initials: D
authors.lastname: Holzinger
authors.forename: J
authors.initials: J
authors.lastname: Roth
authors.forename: D
authors.initials: D
authors.lastname: Föll
authors.forename: T
authors.initials: T
authors.lastname: Niehues
mesh_terms: D001688:Biological Products / Q000627:therapeutic use*; D010505:Familial Mediterranean Fever / Q000188:drug therapy* / Q000235:genetics* / Q000276:immunology; D020022:Genetic Predisposition to Disease / Q000235:genetics*; D005858:Germany; D006801:Humans; D012042:Registries
chemical_list: D001688:Biological Products
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