Department of Nephrology, Erasme Hospital, Université Libre de Bruxelles, Brussels, Belgium.
J Am Acad Dermatol. 2010 Sep;63(3):389-99. doi: 10.1016/j.jaad.2009.10.038. Epub 2010 Jul 8.
Nephrogenic systemic fibrosis (NSF) is a fibrotic disorder occurring in patients with renal dysfunction. Exposure to gadolinium (Gd)-based contrast agents (GBCAs) during renal impairment is associated with development of NSF.
A cross-referenced search of kidney transplantation and radiology databases at a single institution revealed the prevalence of NSF in the transplant population. Clinical records and skin biopsy specimens from 6 patients with kidney transplant given a diagnosis of NSF were reviewed to identify contributing factors.
Between January 1999 and December 2006, NSF was diagnosed in 6 of 705 patients with kidney transplant (0.9%). Renal function was impaired in all patients. Of 33 patients with kidney transplant exposed to GBCAs, 5 (15.2%) developed NSF. Disease onset ranged from 7 days to 11 months after exposure to GBCAs. All 5 patients exposed to GBCAs who developed NSF were also treated with a beta-blocker and clinical improvement was observed with discontinuation. The sixth case NSF appeared unrelated to Gd, without a known exposure, and testing of tissue via mass spectrometry revealed no Gd. Symptoms of NSF in this patient disappeared after administration of darbepoetin was switched from subcutaneous to intravenous injection. One patient with NSF who manifested the highest Gd level in tissue died 22 months after disease onset.
The study represents the retrospective experience of only a single center.
NSF can develop in kidney transplant recipients with altered graft function. In these patients, exposure to GBCAs appears associated with development of NSF. The role of beta-blockers in the course of the disease merits further investigation.
肾源性系统性纤维化（NSF）是一种在肾功能障碍患者中发生的纤维化疾病。在肾功能受损期间暴露于钆（Gd）基造影剂（GBCA）与 NSF 的发展相关。
在一家机构的肾脏移植和放射学数据库中进行交叉参考搜索，揭示了移植人群中 NSF 的患病率。对 6 名被诊断为 NSF 的肾移植患者的临床记录和皮肤活检标本进行了回顾，以确定促成因素。
1999 年 1 月至 2006 年 12 月期间，705 例肾移植患者中诊断出 NSF 6 例（0.9%）。所有患者的肾功能均受损。在接受 GBCA 暴露的 33 例肾移植患者中，有 5 例（15.2%）发生 NSF。疾病发作时间从暴露于 GBCA 后 7 天至 11 个月不等。所有 5 例暴露于 GBCA 并发生 NSF 的患者也接受了β受体阻滞剂治疗，并在停药后观察到临床改善。第六例 NSF 似乎与 Gd 无关，没有已知的暴露，通过质谱法对组织进行检测发现没有 Gd。该患者的 NSF 症状在将达贝泊汀的给药途径从皮下注射改为静脉注射后消失。一名 NSF 患者组织中的 Gd 水平最高，在发病后 22 个月死亡。
该研究仅代表单个中心的回顾性经验。
改变移植物功能的肾移植受者可能会发生 NSF。在这些患者中，暴露于 GBCA 似乎与 NSF 的发展有关。β受体阻滞剂在疾病过程中的作用值得进一步研究。
