Systemic lupus erythematosus and Sjögrens syndrome: Edited by David DaikhActivation of the type I interferon pathway in primary Sjögren's syndrome an updateVakaloglou, Katerina M.a; Mavragani, Clio P.b Author Information aDivision of Genetics, Biomedical Research Foundation, Academy of Athens bDepartment of Experimental Physiology, School of Medicine, University of Athens, Athens, Greece Correspondence to Clio P. Mavragani, MD, Department of Experimental Physiology, School of Medicine, M. Asias 75, Goudi, 11527, Athens, GreeceTel: +30 210 746 2714; fax: +30 210 746 2571; e-mail: [email protected] Current Opinion in Rheumatology: September 2011 - Volume 23 - Issue 5 - p 459-464 doi: 10.1097/BOR.0b013e328349fd30 Buy Metrics Abstract Purpose of review 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. Recent findings 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. Summary 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. © 2011 Lippincott Williams & Wilkins, Inc.