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Interferon pathway in SLE: one key to unlocking the mystery of the disease
  1. Lars Rönnblom and
  2. Dag Leonard
  1. Department of Medical Sciences, Rheumatology, Uppsala University, Uppsala, Sweden
  1. Correspondence to Professor Lars Rönnblom; lars.ronnblom{at}


SLE is characterised by an activation of the interferon (IFN) system, which leads to an increased expression of IFN-regulated genes. The reasons behind the IFN signature in SLE are (1) the existence of endogenous IFN inducers, (2) activation of several IFN-producing cell types, (3) production of many different IFNs, (4) a genetic setup promoting IFN production and (5) deficient negative feedback mechanisms. The consequences for the immune system is a continuous stimulation to an immune response, and for the patient a number of different organ manifestations leading to typical symptoms for SLE. In the current review, we will present the existing knowledge of the IFN system and pathway activation in SLE. We will also discuss how this information can contribute to our understanding of both the aetiopathogenesis and some organ manifestations of the disease. We will put forward some issues that are unresolved and should be clarified in order to make a proper stratification of patients with SLE, which seems important when selecting a therapy aiming to downregulate the IFN system.

  • systemic lupus erythematosus
  • interferon
  • plasmacytoid dendritic cell

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  • Contributors LR and DL wrote and approved the manuscript.

  • Funding The study was supported by the Swedish Rheumatism Association, King Gustaf V’s 80-years Foundation, the Swedish Research Council and the Swedish Society of Medicine (the Ingegerd Johansson donation).

  • Competing interests LR has received a research grant from AstraZeneca and received honoraria for scientific advice from Biogen.

  • Patient consent for publication Not required.

  • Provenance and peer review Commissioned; externally peer reviewed.

  • Data availability statement No additional data are available.

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