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Nephrogenic systemic fibrosis

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Abstract

Nephrogenic systemic fibrosis (NSF) is a dermatological fibrosing disease with potential systemic manifestations resulting in mild to severe symptoms including joint contractures. NSF can be lethal in a subset of cases. It has been recently observed that almost all patients with NSF have had severe renal insufficiency and have received prior administrations of gadolinium-based contrast agents (GBCA). This review will focus on the specific evidence from a retrospective study performed at our center in combination with the general experience available. Conclusions summarize our current understanding of NSF. Recommendations are provided for use of GBCA in clinical MRI practice. Conclusions and recommendations include that the risk of inducing NSF is related to higher-dose administrations of GBCA and that there are differences in risk related to differences in gadolinium-chelate formulation, with the highest relative risk associated with gadodiamide (Omniscan, Nycomed Amersham, GE Healthcare, Piscataway, N.J.).

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The following information came to the author’s attention after submission of his article:

One case of NSF has been associated with the administration of Prohance, one of the more stable macrocyclic gadolinium chelates. In this case the patient was described as having stage 5 severe renal insufficiency on dialysis. A total of six administrations of 32 ml each of ProHance was administered over a 2-year period, representing a total dose normally associated with approximately 10 contrast-enhanced examinations. The suggestion is that cumulative dosing should be considred when evaluating patients with severe renal impairment (stage 4 or 5) prior to considering gadolinium administration and that even the most stable agents may cause NSF at higher doses. Statistical evaluation from a single case requires reservation. A firm conclusion remains that the relative collective incidence of NSF cases reported in association with macrocyclic agents, and certain linear agents, remains so small that it is difficult to measure.

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  1. Department of Radiology, Emory University Hospital, Building A, AT622 1365 Clifton Road, NE, Atlanta, GA, 30322, USA

    Diego R. Martin

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  1. Diego R. Martin
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Correspondence to Diego R. Martin.

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The author discloses that the content of this article will describe use of a medical device or pharmaceutical that is “off-label,” i.e. a use not described on the product’s label.

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Martin, D.R. Nephrogenic systemic fibrosis. Pediatr Radiol 38 (Suppl 1), 125–129 (2008). https://doi.org/10.1007/s00247-007-0589-8

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