title: Type I interferon in the pathogenesis of lupus.
abstract: Investigations of patients with systemic lupus erythematosus have applied insights from studies of the innate immune response to define IFN-I, with IFN-α as the dominant mediator, as central to the pathogenesis of this prototype systemic autoimmune disease. Genetic association data identify regulators of nucleic acid degradation and components of TLR-independent, endosomal TLR-dependent, and IFN-I-signaling pathways as contributors to lupus disease susceptibility. Together with a gene expression signature characterized by IFN-I-induced gene transcripts in lupus blood and tissue, those data support the conclusion that many of the immunologic and pathologic features of this disease are a consequence of a persistent self-directed immune reaction driven by IFN-I and mimicking a sustained antivirus response. This expanding knowledge of the role of IFN-I and the innate immune response suggests candidate therapeutic targets that are being tested in lupus patients.
authors.affiliation: Mary Kirkland Center for Lupus Research, Hospital for Special Surgery, New York, NY 10021; and Rheumatology Division, Department of Medicine, NewYork-Presbyterian/Weill Cornell Medical Center, New York, NY 10065 crowm@hss.edu.
authors.forename: Mary K
authors.initials: MK
authors.lastname: Crow
mesh_terms: D000818:Animals; D005786:Gene Expression Regulation / Q000276:immunology*; D006801:Humans; D007113:Immunity, Innate; D007370:Interferon Type I / Q000276:immunology*; D008180:Lupus Erythematosus, Systemic / Q000097:blood / Q000276:immunology*; D051193:Toll-Like Receptors / Q000276:immunology
chemical_list: D007370:Interferon Type I; D051193:Toll-Like Receptors