Kidney fibrosis, characterized by excessive deposition of extracellular matrix (ECM) that leads to tissue scarring, is the final common outcome of a wide variety of chronic kidney diseases. Rather than being distributed uniformly across the kidney parenchyma, renal fibrotic lesions initiate at certain focal sites in which the fibrogenic niche is formed in a spatially confined fashion. This niche provides a unique tissue microenvironment that is orchestrated by a specialized ECM network consisting of de novo-induced matricellular proteins. Other structural elements of the fibrogenic niche include kidney resident and infiltrated inflammatory cells, extracellular vesicles, soluble factors and metabolites. ECM proteins in the fibrogenic niche recruit soluble factors including WNTs and transforming growth factor-β from the extracellular milieu, creating a distinctive profibrotic microenvironment. Studies using decellularized ECM scaffolds from fibrotic kidneys show that the fibrogenic niche autonomously promotes fibroblast proliferation, tubular injury, macrophage activation and endothelial cell depletion, pathological features that recapitulate key events in the pathogenesis of chronic kidney disease. The concept of the fibrogenic niche represents a paradigm shift in understanding of the mechanism of kidney fibrosis that could lead to the development of non-invasive biomarkers and novel therapies not only for chronic kidney disease, but also for fibrotic diseases of other organs.

肾脏纤维化的特征是导致组织瘢痕形成的细胞外基质 (ECM) 过度沉积，是多种慢性肾脏疾病的最终共同结果。肾纤维化病变不是均匀分布在整个肾实质，而是在某些局灶性部位开始，纤维化生态位以空间受限的方式形成。这个利基提供了一个独特的组织微环境，由一个专门的 ECM 网络协调，该网络由从头诱导的基质细胞蛋白组成。纤维化生态位的其他结构元素包括肾脏驻留和浸润的炎症细胞、细胞外囊泡、可溶性因子和代谢物。纤维化生态位中的 ECM 蛋白从细胞外环境中募集可溶性因子，包括 WNT 和转化生长因子-β，创造独特的促纤维化微环境。使用来自纤维化肾脏的脱细胞 ECM 支架的研究表明，纤维化生态位自主促进成纤维细胞增殖、肾小管损伤、巨噬细胞活化和内皮细胞耗竭，这些病理特征概括了慢性肾脏病发病机制中的关键事件。纤维化生态位的概念代表了对肾纤维化机制理解的范式转变，这可能导致非侵入性生物标志物和新疗法的发展，不仅适用于慢性肾脏疾病，也适用于其他器官的纤维化疾病。巨噬细胞活化和内皮细胞耗竭，这些病理特征概括了慢性肾脏病发病机制中的关键事件。纤维化生态位的概念代表了对肾纤维化机制理解的范式转变，这可能导致非侵入性生物标志物和新疗法的发展，不仅适用于慢性肾脏疾病，也适用于其他器官的纤维化疾病。巨噬细胞活化和内皮细胞耗竭，这些病理特征概括了慢性肾脏病发病机制中的关键事件。纤维化生态位的概念代表了对肾纤维化机制理解的范式转变，这可能导致非侵入性生物标志物和新疗法的发展，不仅适用于慢性肾脏疾病，也适用于其他器官的纤维化疾病。