Department of Dermatology, University of Texas Medical Branch, University of Texas, Galveston, Texas.
School of Medicine, University of Texas Medical Branch, University of Texas, Galveston, Texas.
J Am Acad Dermatol. 2017 Aug;77(2):235-240. doi: 10.1016/j.jaad.2017.02.003. Epub 2017 Mar 18.
Despite multiple therapeutic approaches for nephrogenic systemic fibrosis (NSF), no single treatment has convincingly shown consistent benefit. The most successful outcomes have been associated with recovery of renal function, although evidence remains limited and past studies have been inconclusive.
We sought to investigate whether improvement of renal function via successful transplantation or via return of renal function after acute kidney injury correlates with improvement of NSF, and to further characterize the clinical features and progression of NSF.
A retrospective medical chart review led to the identification of patients (n = 8) diagnosed with NSF who presented to a single academic tertiary referral center over a 15-year period. These 8 patients were contacted by phone to obtain information related to treatment and clinical course of their NSF and renal function. Statistical analysis was performed using Fisher's exact test.
There is a significant correlation (P = .0286) of improved renal function with improvement of NSF. All 4 patients who had improvement of renal function also had improvement of NSF. Two of these patients had end-stage renal disease and a successful kidney transplant, and two had acute kidney injury that resolved. No improvement in NSF was observed without kidney function resolution.
Our study is limited by a small sample size (n = 8) and a retrospective study design, which increased its potential for selection and recall bias.
Improvement of renal function through either transplantation or resolution of acute kidney injury with medical management is significantly associated with improvement of NSF.
尽管针对肾源性系统性纤维化 (NSF) 有多种治疗方法，但没有单一的治疗方法能令人信服地显示出一致的益处。最成功的结果与肾功能的恢复有关，尽管证据仍然有限，过去的研究也没有定论。
我们试图研究肾功能的改善（通过成功的移植或急性肾损伤后肾功能的恢复）是否与 NSF 的改善相关，并进一步描述 NSF 的临床特征和进展。
回顾性病历审查确定了 8 名在 15 年内被诊断为 NSF 并在一家学术性三级转诊中心就诊的患者。通过电话联系这 8 名患者，以获取与他们的 NSF 和肾功能的治疗和临床病程相关的信息。使用 Fisher 精确检验进行统计分析。
肾功能的改善与 NSF 的改善有显著相关性（P =.0286）。所有肾功能改善的 4 名患者也有 NSF 的改善。这 2 名患者患有终末期肾病并成功进行了肾移植，2 名患者患有急性肾损伤并得到解决。没有观察到肾功能未恢复时 NSF 的改善。
我们的研究受到样本量小（n = 8）和回顾性研究设计的限制，这增加了选择和回忆偏倚的可能性。
通过移植或通过药物治疗解决急性肾损伤来改善肾功能与 NSF 的改善显著相关。
