title: Lights and shadows in autoinflammatory syndromes from the childhood and adulthood perspective.
abstract: In a high percentage of cases, the monogenic autoinflammatory syndromes (AIS), caused by subversion in the inflammasome homeostasis leading to cytokine oversecretion and characterized by multiple inflammatory pictures, start in childhood. However, the description of tardive manifestations, veiled phenotypes, and atypical clinical signs beginning in adulthood has been more and more reported in recent times, requiring that many specialists become confident with concepts, details, and management strategies of AIS. Differences between child- and adult-onset syndromes raise the question of whether pathogenic mechanisms might differ when the timetable of AIS onset diverges, but show that carefulness is needed to establish a straightforward diagnosis.
authors.affiliation: Institute of Pediatrics, Fondazione Policlinico Universitario A. Gemelli, Università Cattolica Sacro Cuore, Rome, Italy.
authors.forename: Donato
authors.initials: D
authors.lastname: Rigante
authors.affiliation: Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease, Rheumatology Unit, University of Siena, Policlinico Le Scotte, Viale Bracci 1, 53100, Siena, Italy.
authors.forename: Antonio
authors.initials: A
authors.lastname: Vitale
authors.affiliation: Institute of Pediatrics, Fondazione Policlinico Universitario A. Gemelli, Università Cattolica Sacro Cuore, Rome, Italy.
authors.forename: Marco Francesco
authors.initials: MF
authors.lastname: Natale
authors.affiliation: Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease, Rheumatology Unit, University of Siena, Policlinico Le Scotte, Viale Bracci 1, 53100, Siena, Italy. cantariniluca@hotmail.com.
authors.forename: Luca
authors.initials: L
authors.lastname: Cantarini
mesh_terms: D000328:Adult; D000367:Age Factors; D002648:Child; D056660:Hereditary Autoinflammatory Diseases / Q000175:diagnosis* / Q000276:immunology; D006801:Humans; D007113:Immunity, Innate / Q000276:immunology*; D058847:Inflammasomes / Q000276:immunology*; D013577:Syndrome
chemical_list: D058847:Inflammasomes
keywords: Adult; Autoinflammatory syndromes; Child; Inflammasome