Nephrogenic Systemic Fibrosis (NSF): 10 High-Yield Facts
1.) Patients with NSF may present to the Emergency Department: NSF was first seen
in 1997 and first reported in 2000.1-3 The current generation of medicine
infrequently witnesses the development of an entirely new disease, but this is one
example.
2.) NSF patients have thickened and hardened skin of the extremities and trunk, with
brawny hyperpigmentation; the fibrosis may occur in other areas of skin, and in
deeper tissues (subcutaneous tissue, fascia, muscle, liver, lung, and other tissues).4
3.) NSF has only occurred in patients with renal insufficiency; in this group, the risk
of NSF within days to months after gadolinium exposure is roughly 3%-5% higher doses tend to be most associated with disease occurrence.1
4.) Though treatment with plasmapheresis, photopheresis, and thalidomide have been
attempted, there is no cure for NSF; it is a progressive, disabling, and possibly
fatal condition that can occur at any age, mostly seen in middle-age patients.1
5.) These patients need a deep incisional biopsy or punch biopsy to provide an
adequate pathology sample; light microscopy will demonstrate expansion of the
dermis with fibrosis in the setting of CD34+ fibrocytes – special stains show
increased collagen, mucin, and elastic fiber deposition, and occasionally
gadolinium deposits.4, 5 Pathogenesis might involve inappropriate activation of
tissue-injury response, or increased production and recruitment of fibrocytes.4
6.) Other names used for this condition include Nephrogenic Fibrosing Dermopathy
(NFD)2, scleromyxoedema-like cutaneous disease3, and Hemodialysis-Associated
Systemic Fibrosis (HASF, considered a distracting term and not generally used).6
7.) So far, only association with, and not causation by, gadolinium, has been
demonstrated. Many gadolinium-containing contrast agents exist: Omniscan has
been associated most of the cases of NSF, while ProHance and MultiHance have
been associated with none and one case, respectively. (The MultiHance patient
also received Omniscan 5 days afterward).1, 7, 8
8.) Hemodialysis after gadolinium administration is the fastest way to remove the
chemical from blood, but has not yet been demonstrated to decrease the incidence
or severity of NSF. Though gadolinium is nephrotoxic, generally used doses are
low so this is negligible; metformin usually does not need to be discontinued.4, 9-12
9.) Experts4 recommend GFR to stratify risk for NSF:
a. GFR >30: Generally do not need hemodialysis after the scan; only 2
reported cases of NSF in this GFR range, confounded by acute renal injury
(inaccurate GFR evaluations in this setting)4
b. GFR 15-29: Consider hemodialysis, risk may not outweigh benefit.
c. GFR <15 (FDA’s definition of “chronic severe renal failure” as of 2007)
or hepatorenal syndrome, or perioperative patients for hepatic
transplant: Hemodialyze these patients after scan.
10.)
There is an official registry to contribute patient cases of NSF for national
and international research efforts: http://www.icnfdr.org.
References
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Saitoh T, Hayasaka K, Tanaka Y, Kuno T, Nagura Y. Dialyzability of
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Okada S, Katagiri K, Kumazaki T. Yokoyama H Safety of gadolinium contrast
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