The interferon-alpha signature of systemic lupus erythematosus.

The interferon-alpha signature of systemic lupus erythematosus.
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DOI:
10.1177/0961203310371161
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发表时间:
2010-08
期刊:
影响因子:
2.6
通讯作者:
Pascual V
Pascual V
中科院分区:
医学4区
文献类型:
--
作者:
Obermoser G;Pascual V

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系统性红斑狼疮(SLE)是一种典型的多系统自身免疫性疾病,其中环境和遗传危险因素的相互作用导致随着时间的推移对核抗原的耐受性逐渐丧失,最终导致临床疾病。临床表现的异质性和疾病的不可预测的过程,其特征在于耀斑和缓解,很可能反映了异质性的起源疾病,最终共同的途径导致对核抗原的耐受性丧失。免疫复合物和凋亡物质的清除受损以及自身抗体的产生长期以来被认为是这种疾病的主要致病事件。在过去的十年中,I型干扰素细胞因子家族已被假定在SLE发病机制中发挥核心作用,通过促进反馈回路逐步破坏外周免疫耐受和驱动疾病活动。参与SLE发病机制的关键分子的鉴定不仅将提高我们对这一复杂疾病的理解,而且有助于确定生物干预的新靶点。
Systemic lupus erythematosus (SLE) is a prototypic multisystem autoimmune disorder where interplay of environmental and genetic risk factors leads to progressive loss of tolerance to nuclear antigens over time, finally culminating in clinical disease. The heterogeneity of clinical manifestations and the disease’s unpredictable course characterized by flares and remissions are very likely a reflection of heterogeneity at the origin of disease, with a final common pathway leading to loss of tolerance to nuclear antigens. Impaired clearance of immune complexes and apoptotic material and production of autoantibodies have long been recognized as major pathogenic events in this disease. Over the past decade the type I interferon cytokine family has been postulated to play a central role in SLE pathogenesis, by promoting feedback loops progressively disrupting peripheral immune tolerance and driving disease activity. The identification of key molecules involved in the pathogenesis of SLE will not only improve our understanding of this complex disease, but also help to identify novel targets for biological intervention.
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