Division of Liver Diseases, Mount Sinai School of Medicine, New York, NY 10029, USA.
Sci Transl Med. 2013 Jan 9;5(167):167sr1. doi: 10.1126/scitranslmed.3004700.
Fibrosis, or the accumulation of extracellular matrix molecules that make up scar tissue, is a common feature of chronic tissue injury. Pulmonary fibrosis, renal fibrosis, and hepatic cirrhosis are among the more common fibrotic diseases, which in aggregate represent a huge unmet clinical need. New appreciation of the common features of fibrosis that are conserved among tissues has led to a clearer understanding of how epithelial injury provokes dysregulation of cell differentiation, signaling, and protein secretion. At the same time, discovery of tissue-specific features of fibrogenesis, combined with insights about genetic regulation of fibrosis, has laid the groundwork for biomarker discovery and validation, and the rational identification of mechanism-based antifibrotic drugs. Together, these advances herald an era of sustained focus on translating the biology of fibrosis into meaningful improvements in quality and length of life in patients with chronic fibrosing diseases.
纤维化，或构成疤痕组织的细胞外基质分子的积累，是慢性组织损伤的一个常见特征。肺纤维化、肾纤维化和肝硬化是较为常见的纤维化疾病，它们共同代表了巨大的未满足的临床需求。对组织间纤维化的共同特征的新认识，导致了对上皮损伤如何引发细胞分化、信号和蛋白质分泌失调的更清晰理解。与此同时，对纤维化发生的组织特异性特征的发现，结合对纤维化遗传调控的深入了解，为生物标志物的发现和验证以及基于机制的抗纤维化药物的合理鉴定奠定了基础。这些进展共同预示着一个将纤维化生物学转化为慢性纤维化疾病患者生活质量和寿命显著改善的持续关注时代的到来。
