Division of Nephrology, University of Ottawa, Ottawa, Canada.
Clin Exp Dermatol. 2011 Oct;36(7):763-8. doi: 10.1111/j.1365-2230.2011.04136.x. Epub 2011 Jul 25.
Nephrogenic systemic fibrosis (NSF), previously known as nephrogenic fibrosing dermopathy, is a generalized fibrotic disorder occurring in people with renal failure, following exposure to gadolinium-based contrast agents used to enhance MRI. The cellular elements involved in pathology of NSF include bone-marrow-derived collagen-producing fibrocytes, myofibroblasts and activated macrophages. Mechanisms that have been hypothesized to play a role in the pathogenesis of NSF include upregulation of osteopontin, imbalance between matrix metalloproteinases and tissue inhibitor of metalloproteinase 1, and presence of transforming growth factor-β, nuclear factor κB, decorin and metallothioneins. Gadolinium (both free and chelated) is thought to be a bioactive trigger for NSF. Elucidation of these potential pathomechanisms would be useful for development of targeted therapies for NSF.
肾源性系统性纤维化（NSF），以前称为肾源性纤维性皮肤病，是一种发生于肾衰竭患者的全身性纤维性疾病，与使用钆基造影剂增强 MRI 检查有关。NSF 病理学涉及的细胞成分包括骨髓来源的产生胶原的成纤维细胞、肌成纤维细胞和活化的巨噬细胞。推测与 NSF 发病机制有关的机制包括骨桥蛋白的上调、基质金属蛋白酶与金属蛋白酶组织抑制剂 1 之间的失衡，以及转化生长因子-β、核因子 κB、核心蛋白聚糖和金属硫蛋白的存在。认为钆（游离和螯合形式）是 NSF 的生物活性触发因素。阐明这些潜在的病理机制将有助于开发针对 NSF 的靶向治疗方法。
