Division of Rheumatology, Department of Medicine III, University Clinical Center Carl Gustav Carus, Technical University Dresden, Dresden, Germany.
Clin Immunol. 2013 Jun;147(3):216-22. doi: 10.1016/j.clim.2012.11.012. Epub 2012 Dec 12.
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.
先天免疫系统参与了系统性自身免疫性疾病的发病机制，如系统性红斑狼疮（SLE）或皮肌炎。基于遗传和临床证据，经典途径的补体因子和 Toll 样受体（TLRs）的重要作用已得到充分证实。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）。后者启动正反馈环，驱动自身免疫。最近，发现编码参与 RNA 和 DNA 处理的细胞内酶的基因突变同样会导致家族性寒冷性自身免疫性荨麻疹，并与 SLE 相关。在免疫性疾病连续体中，这些疾病可以介于自身炎症和自身免疫之间，我们建议将该组疾病命名为“自佐剂”，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。
