What is nephrogenic systemic fibrosis (NSF)?

Updated: May 22, 2018
  • Author: Noah S Scheinfeld, JD, MD, FAAD; Chief Editor: Dirk M Elston, MD  more...
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Answer

Nephrogenic systemic fibrosis (NSF), also known as nephrogenic fibrosing dermopathy (NFD), is a disease of fibrosis of the skin and internal organs reminiscent but distinct from scleroderma or scleromyxedema. It is caused by gadolinium exposure used in imaging in patients who have renal insufficiency.

Nephrogenic systemic fibrosis almost always occurs  in patients with renal insufficiency who have had imaging studies (eg, magnetic resonance angiography) with gadolinium, a contrast agent used in imaging studies. Gadolinium can be found in tissue samples of nephrogenic systemic fibrosis, and alternative contrast agents are being sought. [1, 2, 3]

Evidence for a link between nephrogenic systemic fibrosis and gadolinium was first described in a case series of 13 patients, all of whom developed nephrogenic systemic fibrosis after being exposed to gadolinium. [4]  Sometimes articles are published that state a patient with renal impairment developed nephrogenic systemic fibrosis, but these are likely instances when an inadequate history has been taken. [5] One case of nephrogenic systemic fibrosis developed 10 years after gadolinium exposure. [6]  Newer contrast agents like gadobenate dimeglumine carry a lower risk. [7, 8]

Nephrogenic systemic fibrosis resembles scleroderma and eosinophilic fasciitis clinically and scleromyxedema histopathologically. Patients with nephrogenic systemic fibrosis may develop large areas of indurated skin with fibrotic nodules and plaques. Flexion contractures with an accompanying limitation of range of motion also can occur. Although most patients with nephrogenic systemic fibrosis have undergone hemodialysis for renal failure, some have never undergone dialysis and others have received only peritoneal dialysis.

Histopathologically, nephrogenic systemic fibrosis resembles scleromyxedema in that it manifests with a proliferation of dermal fibroblasts and dendritic cells, thickened collagen bundles, increased elastic fibers, and mucin deposition.


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