【摘 要】
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Dear Editor,Systemic sclerosis (scleroderma,SSc) is a chronic disease of connective tissues,and is clinically characterized by persistent fibrosis in the skin a
【机 构】
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Department of Rheumatology and Clinical Immunology, Peking Union Medical College Hospital, Chinese A
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Dear Editor,Systemic sclerosis (scleroderma,SSc) is a chronic disease of connective tissues,and is clinically characterized by persistent fibrosis in the skin as well as in a variety of organs (Katsumoto et al.,2011).The pathogenesis of SSc is complex and involves vasculopathy,autoimmunity and fibrosis.The hallmarks of late-stage SSc are the excessive secretion and accumulation of extracellular matrix (ECM) by aberrantly activated fibroblasts (myofibroblasts) in the skin and intemal organs (Bhattacharyya et al.,2012).Similar to other fibrotic diseases,dysfunction of the affected organs is a common feature and results in high morbidity and significantly increased mortality (Akhmetshina et al.,2012).However,effective anti-fibrotic strategies for the treatment of SSc are not available to date,primarily due to an incomplete understanding of the precise mechanism goveing skin fibroblast activation during SSc progression (Distler and Cozzio,2016).Therefore,identification of novel therapeutic targets for fibrotic-targeted therapy of SSc is of paramount importance.
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