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Nephrogenic systemic fibrosis: an emerging entity

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Abstract

Nephrogenic systemic fibrosis (NSF) is a progressive disorder which has been seen only in patients with chronic kidney disease. It is associated with fibrosis of the skin and connective tissue throughout the body. The skin thickens and becomes hard, rigid, and coarse, which severely restricts movement of the joints. It can also lead to widespread fibrosis of internal organs involving lungs, heart, diaphragm, esophagus, and skeletal muscle, and in some cases may lead to death. Based on case series reports and skin biopsy showing gadolinium (Gd) ions, NSF has been directly linked to Gd contrast exposure given during MRI/MRA. There are over 250 reported cases of NSF worldwide with hundreds still not reported or remaining undiagnosed. Symptoms of NSF appear within 2–75 days, with a mean of 25 days after exposure to Gd contrast. This disorder is not well understood, and more research is needed to obtain information about how Gd causes this condition. Currently there is no effective treatment, so prevention is the only way to avoid this serious illness.

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Authors and Affiliations

  1. Department of Nephrology, University of Toronto, 200 Elizabeth Street, Toronto, ON, Canada, M5G2C4

    Sandip K. Saxena, Manjuri Sharma, Mital Patel & Dimitrios Oreopoulos

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  1. Sandip K. Saxena
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  2. Manjuri Sharma
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Correspondence to Sandip K. Saxena.

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Saxena, S.K., Sharma, M., Patel, M. et al. Nephrogenic systemic fibrosis: an emerging entity. Int Urol Nephrol 40, 715–724 (2008). https://doi.org/10.1007/s11255-008-9361-8

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