Cutaneous changes of nephrogenic systemic fibrosis: Predictor of early mortality and association with gadolinium exposure

Abstract

Objective. Nephrogenic systemic fibrosis (NSF) is a rapidly progressive, debilitating condition that causes cutaneous and visceral fibrosis in patients with renal failure. Little is known about its prevalence or etiology. The aim of this study was to establish the prevalence of NSF and associated risk factors Methods. Two cohorts of patients were recruited from 6 outpatient hemodialysis centers and examined for cutaneous changes of NSF, which were defined using a scoring system based on hyperpigmentation, hardening, and tethering of skin on the extremities. Demographic data were gathered, mortality was followed up prospectively for 24 months, and gadolinium exposure was ascertained for a subgroup of patients in the second cohort.Results. Examination reproducibility was 97% in cohort 1. In cohort 2, 25 (13%) of 186 patients demonstrated cutaneous changes of NSF. Twenty-four-month mortality following examination was 48% and 20% in patients with and those without cutaneous changes of NSF, respectively (adjusted hazard ratio 2.9, 95% confidence interval [95% CI] 1.4-5.9). Cutaneous changes of NSF were observed in 16 (30%) of 54 patients with prior exposure to gadopentetate dimeglumine contrast during imaging studies. Exposure to gadoliniumcontaining contrast was associated with an increased risk of developing cutaneous changes of NSF (odds ratio 14.7, 95% CI 1.9-117.0) compared with nonexposed patients.

Retrospective assessment of prevalence of nephrogenic systemic fibrosis (NSF) after implementation of a new guideline for the use of gadobenate dimeglumine as a sole contrast agent for magnetic resonance examination in renally impaired patients

“…A strong association has been demonstrated between exposure to each of several GBCAs and the onset of NSF (7,11,(15)(16)(17)(18)(19). At our institution, all patients who have developed NSF have had renal impairment and previous exposure to GBCAs.…”

“…It is generally believed that there are multiple risk factors for the development of NSF, among which gadoliniumbased contrast agents (GBCAs) have been suggested to trigger the development of NSF, based on the temporal association between the administration of GBCAs and the development of NSF skin changes (5)(6)(7) and the identification of gadolinium in skin and other organs of patients with NSF (2,8,9). The recent identification of NSF and the association between the use of GBCAs and the development of this debilitating disease has raised serious concerns about the safety of GBCAs in individuals with CKD.…”

Revisiting nephrogenic systemic fibrosis in 6 kidney transplant recipients: A single-center experience

“…Sadowski et al 21 reported an annual incidence of 4.6% in a subpopulation of inpatients with proinflammatory events and decreased renal function. More recently, Todd et al 22 reported an incidence of 13% in a group of hemodialyzed patients from 6 centers.…”

The pitfalls of a purely clinical definition of nephrogenic systemic fibrosis: Comment on the article by Todd et al

“…We read with interest the recent article by Todd and colleagues (1). Although we applaud the efforts of the investigators to elucidate information about this potentially devastating condition, we encourage caution in using the clinical scoring system they describe to define nephrogenic systemic fibrosis (NSF).…”