Nephrogenic systemic fibrosis: Review of 408 biopsy-confirmed cases

Abstract

Nephrogenic systemic fibrosis (NSF) has now been virtually eliminated by the discovery of its association with gadolinium-based contrast agents (GBCAs) and the consequent reduced use of GBCA-enhanced magnetic resonance imaging (MRI) in severe renal failure patients. This review of 408 biopsy-confirmed cases shows how to minimize NSF risk when performing GBCA-enhanced MRI or magnetic resonance angiography. The absence of any NSF cases in patients less than 8 years old or greater than 87 years old suggests that infants and elderly patients are already protected. Limiting GBCA dose to a maximum of 0.1 mMol/kg, dialyzing dialysis patients quickly following GBCA administration, delaying administration of GBCA in acute renal failure until after renal function returns or dialysis is initiated, and avoiding nonionic linear GBCA in renal failure patients, especially when there are pro-inflammatory conditions, appear to have reduced NSF risk to the point where safe GBCA-enhanced MRI is possible in most patients.

Gadolinium and nephrogenic systemic fibrosis: an update

“…However, as MedWatch does not require histopathological confirmation, this is likely an overestimate. The NSF Registry at Yale holds a little more than 360 cases, and a recent review counted 408 biopsy-confirmed cases of NSF in the literature [18]. Confirmed cases of NSF have been reported in patients from 8 years to 87 years old, with a peak incidence at ages 51-60 years.…”

“…As the condition progresses, the skin may take on a woody texture. Joint contractures or reduced range of movement are seen in >60 % of patients [18]. Of 345 patients in the NSF Registry in 2009, almost 10 % were known to be restricted to a wheelchair (ICNSFR http://www.icnsfr.org).…”

“…The reported delay between Gd exposure and NSF presentation varies greatly for individual patients, ranging from a few days to 3 years, with a median of 62 days [18]. The incidence of NSF following Gd administration in renally impaired patients varies between 0 % and 18 %.…”

“…This benefit is seen in patients with chronic renal failure who have undergone successful renal transplantation and in patients whose AKI has resolved [69]. No cases of complete remission have been described in the presence of continued renal impairment [18]. Improvement in cutaneous changes associated with NSF in patients with improving renal function is sufficiently convincing that caution must be applied to the interpretation of results from studies reporting successful treatments for NSF in patients with concomitantly improving renal function.…”

Gadolinium-induced fibrosis is counter-regulated by CCN3 in human dermal fibroblasts: a model for potential treatment of nephrogenic systemic fibrosis

“…Nephrogenic systemic fibrosis (NSF) is a clinical syndrome linked, in individuals with renal failure, to gadolinium-based contrast agent (GBCA) exposure during magnetic resonance imaging (MRI) procedures (Cowper et al 2000;Swartz et al 2003;Grobner 2006;Marckmann et al 2006;Mendoza et al 2006;Khurana et al 2007;Yerram et al 2007;Deo et al 2007;Rydahl et al 2008;Zou et al 2011). The disease has been likened to scleroderma, based on histopathology, which includes the presence of dense, thickened collagen bundles in lesional skin.…”