title: Autoinflammatory diseases in pediatrics.
abstract: Monogenic autoinflammatory syndromes are caused by mutations in protein-coding genes that have a pivotal role in the regulation of the inflammatory response. Due to their genetic nature, most of these syndromes usually begin during childhood. They are clinically characterized by recurrent episodes of systemic inflammation (fever with different clinical manifestations, such as skin rash, serositis or arthritis) associated with elevation of acute phase reactants. During symptom-free intervals, patients achieve clinical well-being and normalize infammatory parameters. Amyloidosis is a serious long-term complication. In this update we will discuss the clinical presentation and therapeutic strategies for these diseases in pediatrics.
authors.affiliation: Servicio de Reumatología, Hospital de Niños Ricardo Gutiérrez, Argentina. smeiorin@hotmail.com
authors.forename: Silvia M
authors.initials: SM
authors.lastname: Meiorin
authors.forename: Graciela
authors.initials: G
authors.lastname: Espada
authors.forename: Carlos
authors.initials: C
authors.lastname: Rosè
mesh_terms: D001327:Autoimmune Diseases / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D002648:Child; D056660:Hereditary Autoinflammatory Diseases / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D006801:Humans; D007249:Inflammation / Q000175:diagnosis / Q000209:etiology / Q000276:immunology* / Q000628:therapy