title: Autoinflammatory syndromes.
abstract: There has been an expansion of the autoinflammatory syndromes due to the discovery of new diseases related to mutations in genes regulating the innate immune system and the knowledge gained from these diseases as applied to more common nongenetic inflammatory conditions. Autoinflammatory syndromes are characterized by unprovoked (or triggered by minor events) recurrent episodes of systemic inflammation involving various body systems, which are often accompanied by fever. Inflammation is mediated by polymorphonuclear and macrophage cells through cytokines, particularly interleukin-1. This article reviews the clinical approach to patients with suspected autoinflammatory syndromes, several of the main and new (mostly genetics) syndromes, advances in treatment, and prognosis.
authors.affiliation: Pediatric Rheumatology Unit, Department of Pediatrics, Shaare Zedek Medical Center, PO Box 3235, Jerusalem 91031, Israel. hashkesp@szmc.org.il
authors.forename: Philip J
authors.initials: PJ
authors.lastname: Hashkes
authors.forename: Ori
authors.initials: O
authors.lastname: Toker
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D002648:Child; D003078:Colchicine / Q000627:therapeutic use; D056587:Cryopyrin-Associated Periodic Syndromes / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D010505:Familial Mediterranean Fever / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D056660:Hereditary Autoinflammatory Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D053590:Interleukin 1 Receptor Antagonist Protein; D054078:Mevalonate Kinase Deficiency / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology; D010019:Osteomyelitis / Q000175:diagnosis / Q000188:drug therapy; D053573:Receptors, Interleukin-1 Type I / Q000037:antagonists & inhibitors; D016896:Treatment Outcome; D050257:Tubulin Modulators / Q000627:therapeutic use
chemical_list: D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D053590:Interleukin 1 Receptor Antagonist Protein; D053573:Receptors, Interleukin-1 Type I; D050257:Tubulin Modulators; D003078:Colchicine