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Innate immune processes in lupus erythematosus.

机译:红斑狼疮的先天免疫过程。

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摘要

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.
机译:先天性免疫系统参与全身性自身免疫性疾病的发病机理,例如全身性红斑狼疮(SLE)或皮肌炎。基于遗传和临床证据,已经很好地确立了经典途径和Toll样受体(TLR)的补体因子的重要作用。免疫复合物激活髓样细胞中的肿瘤坏死因子(TNF)和浆细胞样树突状细胞中的干扰素-α(IFNα)。后者会启动一个正反馈回路,从而驱动自身免疫。最近,已经发现编码RNA和DNA处理中涉及的细胞内酶的基因中的突变同样会导致IFNα的增加,从而引起家族性雪白狼疮,并与SLE有关。在免疫疾病连续体内,这些疾病可以置于自身炎症和自身免疫之间,因此我们为该组提出术语“自动佐剂”,因为这些疾病中先天免疫系统的激活似乎降低了(自身)免疫反应的阈值。

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