Aug 24, 2011

Type I interferon and systemic lupus erythematosus

Journal of Interferon & Cytokine Research : the Official Journal of the International Society for Interferon and Cytokine Research
Keith B Elkon, Vivian V Stone

Abstract

Systemic lupus erythematosus (SLE) is a complex systemic autoimmune disease associated with multiple immunologic abnormalities. Prominent among these is upregulation of type I interferon (IFN)?a powerful immune adjuvant. IFN is, in part, produced in SLE in response to autoantigens in the form of self-nucleic acids and their associated nuclear proteins. Sources of these autoantigens include apoptotic and necrotic cells as well as neutrophils undergoing a specific form of cell death called NETosis. Although plasmacytoid dendritic cells are the main producers of IFN-a, other cells are important regulators of this process. Both genetic and environmental risk factors play a role in the development and pathogenesis of SLE. Further highlighting the importance of IFN, candidate gene and genome-wide association studies have identified a number of genes involved in type I IFN pathways associated with SLE. In this review, 3 monogenic deficiencies that result in lupus-like phenotypes and several polygenic variants that have been consistently associated with SLE are highlighted, and the relationship of these genes to IFN-a production is discussed. Clinical associations of the type I IFN pathway and the use of IFN-blocking agents as therapeu...Continue Reading

  • References68
  • Citations46

Citations

Mentioned in this Paper

Pathogenic Aspects
Biochemical Pathway
Immune System Diseases
Lupus Vulgaris
Absolute Neutrophil Count
Autoimmune Diseases of the Nervous System
Lupus Erythematosus, Systemic
Autoantigens
Interferon-alpha
Interferon Type I

Related Feeds

Autoimmune Diseases

Autoimmune diseases occur as a result of an attack by the immune system on the body’s own tissues resulting in damage and dysfunction. There are different types of autoimmune diseases, in which there is a complex and unknown interaction between genetics and the environment. Discover the latest research on autoimmune diseases here.

Related Papers

Current Opinion in Rheumatology
Keith B Elkon, Alice Wiedeman
The Journal of Immunology : Official Journal of the American Association of Immunologists
Uma SriramStefania Gallucci
Journal of Interferon & Cytokine Research : the Official Journal of the International Society for Interferon and Cytokine Research
T B Niewold
© 2020 Meta ULC. All rights reserved