title: Innate immune processes in lupus erythematosus.
abstract: The innate immune system is involved in the pathogenesis of systemic autoimmune diseases such as systemic lupus erythematosus (SLE) or dermatomyositis. The important role of complement factors of the classical pathway and of Toll like receptors (TLRs) is well established, based on genetic and clinical evidence. Immune complexes activate tumor necrosis factor (TNF) in myeloid cells and interferon-α (IFNα) in plasmacytoid dendritic cells. The latter initiates a positive feedback loop that drives autoimmunity. More recently, mutations in genes encoding intracellular enzymes involved in RNA and DNA handling, which likewise lead to increased IFNα, have been found to cause familial chilblain lupus and to be associated with SLE. Within the immunological disease continuum, these disorders can be placed between autoinflammation and autoimmunity, and we would propose the term autoadjuvant for this group, since the activation of the innate immune system in these diseases appears to lower the threshold for (auto)immune reactions.
authors.affiliation: Division of Rheumatology, Department of Medicine III, University Clinical Center Carl Gustav Carus, Technical University Dresden, Dresden, Germany. martin.aringer@uniklinikum-dresden.de
authors.forename: Martin
authors.initials: M
authors.lastname: Aringer
authors.forename: Claudia
authors.initials: C
authors.lastname: Günther
authors.forename: Min Ae
authors.initials: MA
authors.lastname: Lee-Kirsch
mesh_terms: D000936:Antigen-Antibody Complex / Q000276:immunology; D020274:Autoimmune Diseases of the Nervous System / Q000235:genetics / Q000276:immunology; D015551:Autoimmunity / Q000235:genetics / Q000276:immunology*; D002647:Chilblains / Q000235:genetics / Q000276:immunology; D003165:Complement System Proteins / Q000172:deficiency / Q000235:genetics / Q000276:immunology; D003713:Dendritic Cells / Q000276:immunology* / Q000378:metabolism; D003882:Dermatomyositis / Q000235:genetics / Q000276:immunology* / Q000378:metabolism; D006801:Humans; D007113:Immunity, Innate; D016898:Interferon-alpha / Q000378:metabolism; D008178:Lupus Erythematosus, Cutaneous / Q000235:genetics / Q000276:immunology; D008180:Lupus Erythematosus, Systemic / Q000235:genetics / Q000276:immunology* / Q000378:metabolism; D022423:Myeloid Cells / Q000276:immunology / Q000378:metabolism; D009421:Nervous System Malformations / Q000235:genetics / Q000276:immunology; D051193:Toll-Like Receptors / Q000378:metabolism*; D014409:Tumor Necrosis Factor-alpha / Q000378:metabolism
chemical_list: D000936:Antigen-Antibody Complex; D016898:Interferon-alpha; D051193:Toll-Like Receptors; D014409:Tumor Necrosis Factor-alpha; D003165:Complement System Proteins