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Rheumatology (Oxford). 2017 Oct 1;56(10):1662-1675. doi: 10.1093/rheumatology/kew431.

Type I interferon-mediated autoimmune diseases: pathogenesis, diagnosis and targeted therapy.

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Leeds Teaching Hospitals NHS Trust, NIHR Leeds Biomedical Research Unit.
Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK.


Type I interferons (IFN-Is) are a group of molecules with pleiotropic effects on the immune system forming a crucial link between innate and adaptive immune responses. Apart from their important role in antiviral immunity, IFN-Is are increasingly recognized as key players in autoimmune CTDs such as SLE. Novel therapies that target IFN-I appear effective in SLE in early trials, but effectiveness is related to the presence of IFN-I biomarkers. IFN-I biomarkers may also act as positive or negative predictors of response to other biologics. Despite the high failure rate of clinical trials in SLE, subgroups of patients often respond better. Fully optimizing the potential of these agents is therefore likely to require stratification of patients using IFN-I and other biomarkers. This suggests the unified concept of type I IFN-mediated autoimmune diseases as a grouping including patients with a variety of different traditional diagnoses.

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