The immune pathogenesis of scleroderma: context is everything.

The immune pathogenesis of scleroderma: context is everything.
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DOI:
10.1007/s11926-012-0297-8
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发表时间:
2013-01
影响因子:
5
通讯作者:
Aliprantis, Antonios O.
Aliprantis, Antonios O.
中科院分区:
医学2区
文献类型:
--
作者:
Greenblatt, Matthew B.;Aliprantis, Antonios O.

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The fundamental mechanisms that drive the pathogenesis of Systemic sclerosis (SSc) remain elusive, despite over 50 years of investigation. Here, we review recent progress in the understanding of the immunopathogenesis of SSc. In particular, we consider Interleukin-13 (IL13), and its upstream and downstream pathways, as an example of an immune system-derived mediator that connects to fibrotic and vascular pathology. Emerging data linking pattern recognition receptors and interferon pathways to SSc is also highlighted. Our discussion is framed by genetic data linking the immune system to SSc risk and efforts to map animal models to subsets of patients recently resolved through gene expression profiling. These developments will help build a contextual framework to better understand previous observations and design the next generation of studies that may finally lead to effective treatments.
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