Russell Thomas M, Herzog Erica L, Bucala Richard
Section of Pulmonary and Critical Care Medicine, Department of Medicine, Yale University, New Haven, CT, USA.
Methods Mol Biol. 2012;900:327-46. doi: 10.1007/978-1-60761-720-4_16.
Scleroderma is an autoimmune disease characterized by the progressive and dysregulated accumulation of collagen in the skin and internal organs. Pulmonary complications including interstitial lung disease have emerged as the greatest cause of mortality in this disease. Because treatments are limited, new areas of investigation are sorely needed. An emerging area of interest in this field is a potential role for fibrocytes as biomarkers or mediators of disease. Fibrocytes are monocyte-derived mesenchymal progenitor cells that exhibit features of extracellular matrix production and wound contraction in addition to immunologic functions such as cytokine and chemokine production, antigen presentation, leukocyte trafficking, and modulation of angiogenesis. Fibrocytes could participate in the pathogenesis of scleroderma lung disease through any or all of these functions and may be useful biomarkers of disease activity. This chapter presents protocols that have been developed for the study of fibrocytes obtained from human circulation and tissues. Protocols for the quantification of fibrocytes in murine models also are described, along with discussion of common technical challenges. It is hoped that this information will allow further investigation of the role that fibrocytes might play in Scleroderma-related lung disease and perhaps lead to new areas of study in this difficult-to-treat and deadly disease.
硬皮病是一种自身免疫性疾病，其特征是皮肤和内脏器官中胶原蛋白进行性且失调地积累。包括间质性肺病在内的肺部并发症已成为该疾病死亡的最大原因。由于治疗方法有限，迫切需要新的研究领域。该领域一个新兴的研究热点是纤维细胞作为疾病生物标志物或介质的潜在作用。纤维细胞是单核细胞衍生的间充质祖细胞，除了具有诸如细胞因子和趋化因子产生、抗原呈递、白细胞运输以及血管生成调节等免疫功能外，还表现出细胞外基质产生和伤口收缩的特征。纤维细胞可通过这些功能中的任何一种或全部参与硬皮病肺病的发病机制，并且可能是疾病活动的有用生物标志物。本章介绍了为研究从人体循环和组织中获取的纤维细胞而制定的方案。还描述了在小鼠模型中定量纤维细胞的方案，以及对常见技术挑战的讨论。希望这些信息能让人们进一步研究纤维细胞在硬皮病相关肺病中可能发挥的作用，并可能在这种难以治疗的致命疾病中开辟新的研究领域。
