Department of Internal Medicine I, Division of Cardiology, University Hospital Wuerzburg, Wuerzburg, Germany.
J Cardiovasc Magn Reson. 2012 May 20;14(1):31. doi: 10.1186/1532-429X-14-31.
Nephrogenic Systemic Fibrosis is a rare condition appearing only in patients with severe renal impairment or failure and presents with dermal lesions and involvement of internal organs. Although many cases are mild, an estimated 5% have a progressive debilitating course. To date, there is no known effective treatment thus stressing the necessity of ample prevention measures. An association with the use of Gadolinium based contrast agents (GBCA) makes Nephrogenic Systemic Fibrosis a potential side effect of contrast enhanced magnetic resonance imaging and offers the opportunity for prevention by limiting use of gadolinium based contrast agents in renal failure patients. In itself toxic, Gadolinium is embedded into chelates that allow its safe use as a contrast agent. One NSF theory is that Gadolinium chelates distribute into the extracellular fluid compartment and set Gadolinium ions free, depending on multiple factors among which the duration of chelates exposure is directly related to the renal function. Major medical societies both in Europe and in North America have developed guidelines for the usage of GBCA. Since the establishment of these guidelines and the increased general awareness of this condition, the occurrence of NSF has been nearly eliminated. Giving an overview over the current knowledge of NSF pathobiochemistry, pathogenesis and treatment options this review focuses on the guidelines of the European Medicines Agency, the European Society of Urogenital Radiology, the FDA and the American College of Radiology from 2008 up to 2011 and the transfer of this knowledge into every day practice.
肾源性系统性纤维化是一种罕见病症，仅出现在严重肾功能损害或衰竭的患者中，表现为皮肤损伤和内脏器官受累。虽然许多病例较为轻微，但约有 5%的患者病情呈进行性恶化。迄今为止，尚无已知的有效治疗方法，因此强调了充分预防措施的必要性。与使用基于钆的造影剂（GBCA）有关，使肾源性系统性纤维化成为对比增强磁共振成像的潜在副作用，并通过限制在肾功能衰竭患者中使用基于钆的造影剂来提供预防的机会。钆本身具有毒性，嵌入螯合物中使其可作为造影剂安全使用。一个 NSF 理论是，钆螯合物分布到细胞外液隔室中，并根据多种因素（其中螯合物暴露的持续时间与肾功能直接相关）释放出自由的钆离子。欧洲和北美的主要医学协会都制定了 GBCA 使用指南。自这些指南的建立以及对这种情况的认识提高以来，NSF 的发生已几乎被消除。本文概述了 NSF 的病理生物化学、发病机制和治疗选择的最新知识，重点介绍了 2008 年至 2011 年欧洲药品管理局、欧洲泌尿生殖放射学会、美国食品和药物管理局以及美国放射学会的指南，以及将这些知识转化为日常实践。
