Prasad Srinivasa R, Jagirdar Jaishree
Department of Radiology, University of Texas Health Science Center at San Antonio, San Antonio, TX 78229, USA.
J Comput Assist Tomogr. 2008 Jan-Feb;32(1):1-3. doi: 10.1097/RCT.0b013e31805d08ee.
Nephrogenic systemic fibrosis (NSF) is a rare, idiopathic systemic fibrosing disorder that is predominantly characterized by a clinicopathologically distinct symmetric dermopathy and debilitating joint contractures. The condition affects patients with renal insufficiency and appears to show improvement with prompt restoration of renal function. Although NSF was initially reported in the United States as nephrogenic fibrosing dermopathy, it has subsequently been described in patients in Europe and Asia. More than 215 cases of NSF have been reported in the nephrogenic fibrosing dermopathy/NSF registry that is currently maintained at the Yale University. Recent reports suggest a possible etiologic link between systemic administration of gadolinium-containing magnetic resonance imaging contrast agents in patients with renal insufficiency and the development of NSF.
肾源性系统性纤维化(NSF)是一种罕见的特发性系统性纤维化疾病,其主要特征为临床上和病理上独特的对称性皮肤病以及使人衰弱的关节挛缩。该病影响肾功能不全患者,肾功能迅速恢复后病情似乎会有所改善。尽管NSF最初在美国被报道为肾源性纤维化皮肤病,但随后在欧洲和亚洲的患者中也有描述。耶鲁大学目前维护的肾源性纤维化皮肤病/NSF登记处已报告了215多例NSF病例。最近的报告表明,肾功能不全患者全身使用含钆磁共振成像造影剂与NSF的发生之间可能存在病因学联系。