SYSTEMIC LUPUS ERYTHEMATOSUS AND SJöGREN SYNDROME: Edited by Mariana J. KaplanAn update on the role of type I interferons in systemic lupus erythematosus and Sjögren's syndromeThorlacius, Gudny Ellaa; Wahren-Herlenius, Mariea; Rönnblom, LarsbAuthor Information aRheumatology Unit, Department of Medicine, Karolinska Institutet, Stockholm bDepartment of Medical Sciences, Science for Life Laboratory, Rheumatology, Uppsala University, 751 85 Uppsala, Sweden Correspondence to Lars Rönnblom, Department of Medical Sciences, Science for Life Laboratory, Rheumatology, Uppsala University, Uppsala, Sweden. E-mail: [email protected] Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal's Website (www.co-rheumatology.com). Current Opinion in Rheumatology: September 2018 - Volume 30 - Issue 5 - p 471-481 doi: 10.1097/BOR.0000000000000524 Buy SDC Metrics Abstract Purpose of review Systemic lupus erythematosus (SLE) and primary Sjögren's syndrome (pSS) share several clinical and laboratory features, including an overexpression of type I interferon (IFN) regulated genes. The genetic background to this IFN signature and the role of the type I IFN system in the disease process have been partly clarified. Here, we summarize the latest information concerning the type I IFN system in both diseases. Recent findings A number of gene variants in the type I IFN signalling pathways associate with an increased risk for both SLE and pSS in several ethnicities. The function of some risk gene variants has been elucidated, as well as the importance of epigenetic changes in type I IFN regulated genes. MicroRNA-451 and miR-302d have been shown to target IFN regulatory factor 8 and 9, suggesting that noncoding RNAs can control the IFN system. A prominent type I IFN activation is related to several disease manifestations, and in SLE to a more severe disease phenotype. Phase II studies in SLE suggest beneficial effects of blocking the type I IFN receptor. Summary The activated type I IFN system in SLE and pSS has a strong genetic component, is important in the disease etiopathogenesis and can be targeted. Copyright © 2018 Wolters Kluwer Health, Inc. All rights reserved.