title: [Autoinflammatory syndromes: inborn errors of natural immunity].
abstract: The notion of autoinflammatory diseases delineates a heterogenous group of genetic pathologies characterized by spontaneous periodic systemic inflammation in the absence of infectious or autoimmune causes. The general hypothesis is that the innate immune response in these patients is wrongly tuned, being either too sensitive to minor stimuli or turned off too late. Clinical pictures of these disorders are characterized by high spiking fever associated with involvement of musculo-skeletal system, tegumentary apparatus and serosas. Although inflammatory syndromes are considered rare, they may represent a model in order to unravel some aspects of the innate immune system and of the inflammatory cascade.
authors: Sezione di Reumatologia, Dipartimento di Medicina Clinica e Sperimentale, Università, Ferrara. teocolina@libero.it
authors: Matteo
authors: M
authors: Colina
authors: Marcello
authors: M
authors: Govoni
authors: Francesco
authors: F
authors: De Leonardis
authors: Simone
authors: S
authors: Bernardi
authors: Stefania
authors: S
authors: Volpinari
authors: Gessica
authors: G
authors: Limpido
authors: Francesco
authors: F
authors: Trotta
mesh_terms: D000328:Adult; D001327:Autoimmune Diseases / Q000453:epidemiology / Q000235:genetics / Q000276:immunology; D002648:Child; D016207:Cytokines / Q000378:metabolism; D005334:Fever / Q000209:etiology; D006801:Humans; D007113:Immunity, Innate / Q000235:genetics; D007249:Inflammation / Q000145:classification / Q000453:epidemiology / Q000235:genetics / Q000276:immunology*; D018448:Models, Immunological; D009140:Musculoskeletal Diseases / Q000209:etiology; D013577:Syndrome
chemical_list: D016207:Cytokines