Nephrogenic systemic fibrosis

A 15-year retrospective study at a single tertiary care center

Janice Wilson, Kristyna Gleghorn, Quincy Seigel, Brent Kelly

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

Background: 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. Objective: 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. Methods: 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. Results: 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. Limitations: 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. Conclusion: Improvement of renal function through either transplantation or resolution of acute kidney injury with medical management is significantly associated with improvement of NSF.

Original languageEnglish (US)
JournalJournal of the American Academy of Dermatology
DOIs
StateAccepted/In press - 2017

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Nephrogenic Fibrosing Dermopathy
Tertiary Care Centers
Retrospective Studies
Kidney
Acute Kidney Injury
Transplantation
Selection Bias
Recovery of Function
Sample Size
Chronic Kidney Failure
Therapeutics

Keywords

  • Gadolinium
  • Kidney disease
  • Kidney transplant
  • Nephrogenic systemic fibrosis

ASJC Scopus subject areas

  • Dermatology

Cite this

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title = "Nephrogenic systemic fibrosis: A 15-year retrospective study at a single tertiary care center",
abstract = "Background: 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. Objective: 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. Methods: 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. Results: 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. Limitations: 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. Conclusion: Improvement of renal function through either transplantation or resolution of acute kidney injury with medical management is significantly associated with improvement of NSF.",
keywords = "Gadolinium, Kidney disease, Kidney transplant, Nephrogenic systemic fibrosis",
author = "Janice Wilson and Kristyna Gleghorn and Quincy Seigel and Brent Kelly",
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language = "English (US)",
journal = "Journal of the American Academy of Dermatology",
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T2 - A 15-year retrospective study at a single tertiary care center

AU - Wilson, Janice

AU - Gleghorn, Kristyna

AU - Seigel, Quincy

AU - Kelly, Brent

PY - 2017

Y1 - 2017

N2 - Background: 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. Objective: 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. Methods: 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. Results: 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. Limitations: 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. Conclusion: Improvement of renal function through either transplantation or resolution of acute kidney injury with medical management is significantly associated with improvement of NSF.

AB - Background: 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. Objective: 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. Methods: 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. Results: 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. Limitations: 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. Conclusion: Improvement of renal function through either transplantation or resolution of acute kidney injury with medical management is significantly associated with improvement of NSF.

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KW - Kidney transplant

KW - Nephrogenic systemic fibrosis

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