title: Activation of the type I interferon pathway in primary Sjögren's syndrome: an update.
abstract: OBJECTIVE
  Recent advances suggest type I interferon (IFN) pathway as an emerging mediator of systemic autoimmunity. This review aims to summarize the latest developments in the biology of type I IFN pathway and its contributory role in the pathogenesis of autoimmune disorders with a particular focus on Sjögren's syndrome.
  
  
  RESULTS
  Increased circulating type I IFN levels along with upregulated type I IFN inducible genes in salivary gland tissues, peripheral blood and mononuclear cells suggest activation of type I IFN pathway in Sjögren's syndrome. Additional regulatory mechanisms and novel potential suppressors of type I IFN production provide new insights into disease pathogenesis, pointing to type I IFN system as a potential new therapeutic target.
  
  
  CONCLUSIONS
  Compelling evidence suggests type I IFN as a key player in pathogenesis of Sjögren's syndrome and an attractive potential therapeutic target. Meticulous stratification of patient subgroups characterized by activation of type I IFN pathway should be performed in carefully designed translational studies.
authors.affiliation: Division of Genetics, Biomedical Research Foundation, Academy of Athens, Athens, Greece.
authors.forename: Katerina M
authors.initials: KM
authors.lastname: Vakaloglou
authors.forename: Clio P
authors.initials: CP
authors.lastname: Mavragani
mesh_terms: D006801:Humans; D056747:Immunomodulation; D007370:Interferon Type I / Q000096:biosynthesis* / Q000097:blood; D011110:Polymorphism, Genetic; D012469:Salivary Glands / Q000276:immunology; D015398:Signal Transduction / Q000276:immunology; D012859:Sjogren's Syndrome / Q000235:genetics / Q000276:immunology* / Q000628:therapy
chemical_list: D007370:Interferon Type I