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首页> 外文期刊>Journal of dermatological science >Pathogenesis of systemic sclerosis: altered B cell function is the key linking systemic autoimmunity and tissue fibrosis.
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Pathogenesis of systemic sclerosis: altered B cell function is the key linking systemic autoimmunity and tissue fibrosis.

机译：全身性硬化症的发病机制：B细胞功能的改变是将全身性自身免疫与组织纤维化联系起来的关键。

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Systemic sclerosis (SSc) is characterized by autoimmunity and tissue fibrosis. There is a close association between specific autoantibodies and clinical features in patients with SSc. A number of studies have demonstrated that various cytokines, such as transforming growth factor-beta, modulate the synthesis of extracellular matrix by fibroblasts. However, it is not clear as to how autoimmunity and tissue fibrosis interact with each other. Recent studies have revealed that B cells play a critical role in various systemic autoimmune disorders. CD19 is a central regulator of B cell signaling threshold, and B cells from SSc patients exhibit an increased expression of CD19 that induces SSc-specific autoantibody production in transgenic mice. Furthermore, SSc patients have intrinsic B cell abnormalities characterized by decreased but activated memory B cells, which is possibly due to CD19 overexpression. Similarly, B cells from a tight-skin mouse, a model of SSc, show augmented CD19 signaling and chronic B cell activation. Remarkably, CD19 loss results in inhibition of chronic B cell hyper-reactivity and elimination of autoantibody production, which is associated with improvement in skin fibrosis and a parallel decrease in IL-6 production by B cells. Therefore, augmented cytokine production by B cells is a potential candidate for the induction of skin sclerosis. Alternatively, B cells may influence tissue fibrosis by regulating T cell activation and cytokine production through their antigen-presenting and co-stimulatory abilities. Thus, altered B cell function may result in tissue fibrosis, as well as autoimmunity, in SSc.
机译：系统性硬化症（SSc）的特征是自身免疫和组织纤维化。 SSc患者的特异性自身抗体与临床特征之间有着密切的联系。大量研究表明，多种细胞因子（例如转化生长因子-β）可调节成纤维细胞对细胞外基质的合成。但是，关于自身免疫和组织纤维化如何相互作用尚不清楚。最近的研究表明，B细胞在各种系统性自身免疫性疾病中起关键作用。 CD19是B细胞信号传导阈值的中央调节剂，来自SSc患者的B细胞显示出CD19的表达增加，从而诱导转基因小鼠产生SSc特异性自身抗体。此外，SSc患者具有固有的B细胞异常，其特征是记忆B细胞减少但被激活，这可能是由于CD19过表达引起的。同样，来自紧密皮肤小鼠（SSc模型）的B细胞显示出增强的CD19信号传导和慢性B细胞活化。值得注意的是，CD19的丧失导致慢性B细胞反应过度的抑制和自身抗体产生的消除，这与皮肤纤维化的改善和B细胞IL-6产生的平行降低有关。因此，由B细胞增加的细胞因子产生是诱导皮肤硬化的潜在候选者。或者，B细胞可通过其抗原呈递和共刺激能力来调节T细胞活化和细胞因子产生，从而影响组织纤维化。因此，改变的B细胞功能可导致SSc中的组织纤维化以及自身免疫。

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