What is the prevalence of nephrogenic systemic fibrosis (NSF) in the US?

Updated: Sep 18, 2020
  • Author: Shawn Cowper, MD; Chief Editor: Dirk M Elston, MD  more...
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Nephrogenic systemic fibrosis is an uncommon condition. Since 1997, hundreds of cases have been reported to the NFD Registry. Few cases have occurred since guidelines have been instituted by the ACR, FDA, and EU regulatory authorities; however, this author has heard at meetings that a few sporadic cases have occurred. These cases do not seem to have been reported the NSF Registry at Yale. That is, even with guidelines in place, in 2010, 2011, 2012, 2013, a handful of new cases of were reported but many fewer than before gadolinium was defined as the cause. Some of these cases likely involve exposure to gadolinium used before the guidelines were put in place and involve delayed onset of the disease.

Deo et al [32] studied a population of patients with end-stage renal disease in and around Bridgeport, Connecticut over an 18-month period. The incidence of nephrogenic systemic fibrosis was 4.3 cases per 1000 patient-years. Each radiologic study using gadolinium presented a 2.4% risk for developing nephrogenic systemic fibrosis.

Todd et al [33] found that exposure to gadolinium-containing contrast was associated with an increased risk of developing cutaneous changes of nephrogenic systemic fibrosis (odds ratio, 14.7; 95% confidence interval, 1.9-117.0) compared with patients who were not exposed to gadolinium.

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