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Eloranta ML, Rönnblom L
J. Mol. Med. 94 (10) 1103-1110 [2016-10-00; online 2016-04-20]
Patients with systemic lupus erythematosus (SLE) have an increased expression of type I interferon (IFN)-regulated genes (an IFN signature), which is caused by an ongoing production of type I IFNs by plasmacytoid dendritic cells (pDCs). The reasons behind the continuous IFN production in SLE are the presence of self-derived IFN inducers and a lack of negative feed-back signals that downregulate the IFN response. In addition, several cells in the immune system promote the IFN production by pDCs and gene variants in the type I IFN signaling pathway contribute to the IFN signature. The type I IFNs act as an immune adjuvant and stimulate T cells, B cells, and monocytes, which all play an important role in the loss of tolerance and persistent autoimmune reaction in SLE. Consequently, new treatments aiming to inhibit the activated type I IFN system in SLE are now being developed and investigated in clinical trials.
PubMed 27094810
DOI 10.1007/s00109-016-1421-4
Crossref 10.1007/s00109-016-1421-4
pii: 10.1007/s00109-016-1421-4
pmc: PMC5052287