Anti-interferon alpha treatment in SLE

Clin Immunol. 2013 Sep;148(3):303-12. doi: 10.1016/j.clim.2013.02.013. Epub 2013 Mar 1.


Several studies in the last decade have highlighted the role of the type I interferon (IFN-I) pathway, and particularly interferon alpha (IFNα) in SLE pathogenesis. As a result, a multitude of potential treatments targeting IFNα have emerged in the last few years, a few of which have already completed phase II clinical trials. Some of the treatment strategies have focused on blocking IFNα or its receptor and others the plasmacytoid dendritic cell (pDC), which is the principal IFNα producing cell. In this review, we will discuss the evidence supporting a pathogenic role of IFNα and pDC in SLE, provide an update on the current status of these therapeutic strategies, and discuss the potential advantages and disadvantages of each therapeutic approach.

Keywords: AE; ANA; APECED; B-cell activation factor; BAFF; BDCA2; BILAG index; British Isles Lupus Assessment Group index; CCL2; CXCL10; Clinical trial; DC; DNAseI; Fc gamma receptor IIa, GC, glucocorticoids; FcγRIIa; GMCSF; GRIP1; IFN-I; IFN-regulated genes; IFN-regulatory factor; IFN-stimulated response element; IFNAR; IFNα; IFNα Kinoid; IFNα receptor; IFNα-K; IL; ILT7; IRAK; IRF; IRG; ISRE; IV; Ig; Ig-like transcript 7; Interferon alpha; KLH; MHC; Major Histocompatibility Complex; MyD88; NET; NFκB; NK; ODN; PBMC; PD; PI3Kδ; Plasmacytoid dendritic cells; RBP; RDBPC; RNA binding proteins; RT-qPCR; SAE; SC; SLE; SLE response index; SRI; STAT; Systemic lupus erythematosus; T helper 1; TLR; TNF alpha-induced protein 3; TNFAIP3; TNIP1; TRAF; TYK2; Th1; Toll-like receptors; UV; adverse event(s); antinuclear antibodies; autoimmune polyendocrinopathy candidiasis ectodermal dystrophy; blood DC antigen 2; chemokine (C–C) motif ligand 2; chemokine (C–X–C) motif ligand 10; dendritic cell; deoxyribonuclease I; double stranded DNA; dsDNA; glucocorticoid receptor-interacting protein 1; granulocyte/macrophage colony-stimulating factor; immunoglobulin; interferon alpha; interleukin; interleukin receptor associated kinase; intravenously; keyhole limpet haemocyanin; mAb; monoclonal antibodies; myeloid differentiation factor 88; natural killer; neutrophil extracellular trap; nuclear factor kappa B; oligodeoxynucleotides; pDC; peripheral blood mononuclear cells; pharmacodynamic; phosphatidylinositol-3 kinase δ; plasmacytoid dendritic cell; randomized double-blind placebo controlled; real-time quantitative polymerase chain reaction; serious adverse event (s); signal transducer and activator of transcription; subcutaneously; systemic lupus erythematosus; tumor necrosis factor receptor-associated factor; tumor necrosis factor α-induced protein 3-interacting protein 1; type I interferon(s); tyrosine kinase 2; ultraviolet.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Dendritic Cells / physiology
  • Genetic Predisposition to Disease
  • Humans
  • Interferon-alpha / antagonists & inhibitors*
  • Interferon-alpha / physiology
  • Lupus Erythematosus, Systemic / drug therapy*
  • Lupus Erythematosus, Systemic / etiology
  • Lupus Erythematosus, Systemic / genetics
  • Lupus Erythematosus, Systemic / immunology
  • Receptor, Interferon alpha-beta / antagonists & inhibitors
  • Signal Transduction


  • Interferon-alpha
  • Receptor, Interferon alpha-beta