Nephrogenic systemic fibrosis: a systemic fibrosing disease resulting from gadolinium exposure

Best Pract Res Clin Rheumatol. 2012 Aug;26(4):489-503. doi: 10.1016/j.berh.2012.07.008.

Abstract

Nephrogenic systemic fibrosis (NSF) is an iatrogenic fibrosing disorder that primarily affects individuals with chronic kidney disease (CKD) following exposure to gadolinium-based contrast agents (GBCAs) during imaging procedures. NSF is characterised by skin thickening, tethering and hyperpigmentation; flexion contractures of joints; and extracutaneous fibrosis. This article reviews the history, clinical manifestations, epidemiology, histopathology and pathophysiology of this disabling disease.

Publication types

  • Research Support, N.I.H., Extramural
  • Review

MeSH terms

  • Contracture / chemically induced
  • Contrast Media / adverse effects*
  • Contrast Media / chemistry
  • Diagnosis, Differential
  • Gadolinium / adverse effects*
  • Gadolinium / chemistry
  • Humans
  • Hyperpigmentation / chemically induced
  • Nephrogenic Fibrosing Dermopathy / chemically induced*
  • Nephrogenic Fibrosing Dermopathy / complications
  • Nephrogenic Fibrosing Dermopathy / diagnosis
  • Nephrogenic Fibrosing Dermopathy / pathology
  • Nephrogenic Fibrosing Dermopathy / therapy
  • Renal Insufficiency, Chronic / complications*
  • Risk Factors
  • Skin / pathology

Substances

  • Contrast Media
  • Gadolinium