title: [Type I interferonopathies. Systemic inflammatory diseases triggered by type I interferons].
abstract: Type I interferons mediate immune defense against viral infections. The induction of type I interferons has stimulating and modulating effects on the innate and adaptive immune systems thereby reducing tolerance against self-antigens. Genetic defects that result in an inadequate activation of the type I interferon system can cause a group of inflammatory disorders, which are collectively referred to as type I interferonopathies. While the clinical spectrum of type I interferonopathies is broad and heterogeneous, neurological and cutaneous symptoms are the most frequent manifestations. Some clinical and genetic features of type I interferonopathies are shared by multifactorial diseases, such as systemic lupus erythematosus and systemic vasculitis. Advances in understanding the disease mechanisms underlying type I interferonopathies have pinpointed novel targets for therapeutic interventions.
authors.affiliation: Klinik für Dermatologie, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden, Fetscherstr. 74, 01307, Dresden, Deutschland. claudia.guenther@uniklinikum-dresden.de.
authors.forename: C
authors.initials: C
authors.lastname: Günther
authors.affiliation: Klinik für Dermatologie, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden, Fetscherstr. 74, 01307, Dresden, Deutschland.
authors.forename: F
authors.initials: F
authors.lastname: Schmidt
authors.affiliation: Klinik für Kinder- und Jugendmedizin, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden, Dresden, Deutschland.
authors.forename: N
authors.initials: N
authors.lastname: König
authors.affiliation: Klinik für Kinder- und Jugendmedizin, Medizinische Fakultät Carl Gustav Carus, Technische Universität Dresden, Dresden, Deutschland.
authors.forename: M A
authors.initials: MA
authors.lastname: Lee-Kirsch
mesh_terms: D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000276:immunology* / Q000628:therapy; D004198:Disease Susceptibility / Q000276:immunology; D056660:Hereditary Autoinflammatory Diseases / Q000175:diagnosis* / Q000276:immunology* / Q000628:therapy; D054884:Host-Pathogen Interactions / Q000235:genetics / Q000276:immunology*; D006801:Humans; D007370:Interferon Type I / Q000235:genetics / Q000276:immunology*; D035583:Rare Diseases / Q000175:diagnosis / Q000276:immunology / Q000628:therapy
chemical_list: D007370:Interferon Type I
keywords: Autoimmunity; Autoinflammation; Immune system; Monogenic diseases; Systemic lupus erythematosus