Cases Of Nephrogenic Systemic Fibrosis Research Articles

Nephrogenic Systemic Fibrosis in Patients With Chronic Kidney Disease Who Received Gadopentetate Dimeglumine

To determine the prevalence of nephrogenic systemic fibrosis (NSF) in patients with chronic kidney disease (CKD) who have received gadopentetate dimeglumine. We retrospectively studied all patients who underwent contrast-enhanced magnetic resonance imagings (CE-MRIs) between January 1, 2004 and May 31, 2007, in the Kaiser Permanente Medical Care Program, a managed care organization providing care for more than 3.3 million residents in Northern California. All patients received gadopentetate dimeglumine. We used 4 methods to discover cases of NSF: review of pathology slides, review of coded diagnosis, review of visits to dermatologists and rheumatologists, and surveys of physicians. During the study period 115,252 CE-MRIs were performed, including 676 in 530 patients receiving dialysis (92% on chronic dialysis and 8% on acute dialysis) and 3,423 in 2,862 patients with elevated serum creatinine levels at the time of gadolinium chelate administration. One dialysis patient had a definite diagnosis of NSF. In 3 additional patients, 1 on chronic dialysis and 2 with CKD, NSF could not be ruled out. The prevalence of NSF in patients with CKD who received gadopentetate dimeglumine is lower than previously reported in patients who have received less stable formulations of gadolinium chelates. The prevalence of NSF in our patient population is likely underreported as all patients were not individually examined and histology was not available in the majority of patients. Furthermore, the prevalence is likely effected by the lower average dose and frequency of gadolinium chelate administration in this study compared with previous reports in the literature.

Ultrasmall superparamagnetic iron oxides (USPIOs): a future alternative magnetic resonance (MR) contrast agent for patients at risk for nephrogenic systemic fibrosis (NSF)?

Gadolinium (Gd) based contrast agents (GBCAs) in magnetic resonance imaging (MRI) are used in daily clinical practice and appear safe in most patients; however, nephrogenic systemic fibrosis (NSF) is a recently recognized severe complication associated with GBCAs. It affects primarily patients with renal disease, such as stage 4 or 5 chronic kidney disease (CKD; glomerular filtration rate <30 ml/min per 1.73 m(2)), acute kidney injury, or kidney and liver transplant recipients with kidney dysfunction. Contrast-enhanced MRI and computed tomography (CT) scans provide important clinical information and influence patient management. An alternative contrast agent is needed to obtain adequate imaging results while avoiding the risk of NSF in this vulnerable patient group. One potential alternative is ultrasmall superparamagnetic iron oxide (USPIO) nanoparticles, which provide enhancement characteristics similar to GBCAs. We review our experience in approximately 150 patients on the potential benefits of the USPIOs ferumoxtran-10 and ferumoxytol. We focus on central nervous system (CNS) MRI but also review imaging of other vascular beds. Safety studies, including USPIO administration (ferumoxytol) as iron supplement therapy in CKD patients on and not on dialysis, suggest that decreased kidney function does not alter the safety profile. We conclude that for both CNS MR imaging and MR angiography, USPIO agents like ferumoxytol are a viable option for patients at risk for NSF.

Nephrogenic Systemic Fibrosis: Incidence, Associations, and Effect of Risk Factor Assessment—Report of 33 Cases

To describe the presentation and clinical course of patients with nephrogenic systemic fibrosis (NSF) at a large acute-care hospital, to evaluate the overall incidence of NSF, and to assess the effect of a hospital-wide policy regarding gadolinium-based contrast agent (GBCA) use on NSF incidence. A review of all cases of NSF observed at an institution from 2003 to 2008 was conducted. This HIPAA-compliant study was approved by the institutional review board. The informed consent requirement was waived. Demographics, medical history, and associated conditions were recorded. Radiologic procedures were evaluated if they were performed within 1 year prior to NSF onset. GBCA use was assessed by checking the electronic database for each procedure. The incidence of NSF was compared before and after implementation of an institutional policy designed to assess risk of NSF prior to GBCA use. All 33 patients with NSF (mean age, 49 years; age range, 15-78 years) had advanced renal failure (estimated glomerular filtration rate < 15 mL/min/1.73 m(2)) when the GBCA was injected. Twenty-six patients had severe chronic or end-stage renal disease, and seven had acute renal failure. The mean interval between contrast material injection and NSF onset was 29 days +/- 25 (standard deviation) (range, 4-112 days). The overall incidence of NSF was 36.5 cases per 100,000 gadolinium-enhanced magnetic resonance (MR) examinations between 2003 and 2006 and four cases per 100,000 gadolinium-enhanced MR examinations between 2007 and 2008 after screening for NSF risk was instituted (Fisher exact test, P = .001). Five patients developed NSF in the peritransplant period, and four underwent a catheter-based radiographic procedure with administration of a GBCA. Common associations of GBCA MR imaging and NSF were acute and severe chronic renal failure and liver or renal transplantation. Screening procedures performed before MR imaging to determine which patients were at risk of developing NSF appear to reduce the incidence of this complication and further support the belief that NSF is associated with GBCA administration.

Nephrogenic Systemic Fibrosis Associated With Gadoversetamide Exposure: Treatment With Sodium Thiosulfate

Nephrogenic systemic fibrosis (NSF) is a debilitating fibrosing disorder of patients with kidney disease that is associated with gadolinium-based contrast exposure. Most cases are linked to gadodiamide. Gadoversetamide, an agent with chelate characteristics similar to gadodiamide, has rarely been described to cause NSF. With the exception of normalization of kidney function, there are no consistently effective therapies for patients with NSF. We describe 3 cases of NSF in patients with end-stage renal disease after gadolinium-based contrast exposure. Two patients received gadoversetamide and the third received gadodiamide. All 3 patients were treated early in their disease course with intravenous sodium thiosulfate and responded with improved skin changes and joint mobility.

Gadolinium-based contrast agents and nephrogenic systemic fibrosis: a systematic review and meta-analysis

In the past decade, more than 200 cases of nephrogenic systemic fibrosis (NSF) have been identified, primarily among patients with advanced kidney disease. Multiple studies have suggested an association between gadolinium-based contrast agents (GBCAs) and NSF. We performed a systematic review and meta-analysis to examine this potential association. A systematic review of studies examining the association between any GBCA and NSF was performed. A search for controlled studies was conducted in MEDLINE, EMBASE and the Cochrane Central Register of Controlled Trials. If controlled data for a GBCA was not available, we searched for case reports and series. Relevant data were extracted and meta-analyses were performed. Seven of 144 identified studies met inclusion criteria; gadodiamide was the sole or predominant GBCA in four of these; one study exclusively examined gadopentetate. Other GBCAs were not specifically examined in controlled or uncontrolled studies. Meta-analysis of controlled trials demonstrated a significant association between GBCA exposure and NSF [odds ratio (OR) 26.7; 95% confidence interval (CI) 10.3-69.4] and gadodiamide and NSF (OR 20.0; 95% CI 3.7-107.8). Examination of the evidence using established criteria suggested that this association was causal. The current state of evidence suggests an association and potentially causal link between the use of GBCAs and the development of NSF among patients with advanced kidney disease. Additional study is warranted to clarify the potential association of GBCAs other than gadodiamide with NSF.

Incidence of Nephrogenic Systemic Fibrosis at Two Large Medical Centers

To determine the incidence and associated risk factors of nephrogenic systemic fibrosis (NSF) in patients who undergo gadolinium-based contrast agent (GBCA)-enhanced magnetic resonance (MR) imaging. Institutional review board approval was obtained for retrospective review of the medical records from two hospitals to identify all cases of biopsy-confirmed NSF and all patients administered a GBCA from January 1, 1997, to June 30, 2007. Informed patient consent was not required. The incidence of NSF was calculated for patients who received a standard dose of GBCA, patients who received a high dose, and subgroups of patients with renal impairment. Fifteen patients developed NSF after gadolinium-enhanced MR imaging. All of them had an estimated glomerular filtration rate (eGFR) lower than 30 mL/min, and 11 had acute renal failure or acute deterioration of chronic renal failure. The incidence of NSF after gadolinium-enhanced MR imaging without screening for renal function was zero of 74,124 patients with the standard dose of GBCA and 15 (0.17%) of 8997 patients with the high dose (P < .001). The NSF incidence associated with a high dose of GBCA increased to 0.4% in patients in a chronic hemodialysis program and to 8.8% in those who had an eGFR lower than 15 mL/min but were not undergoing hemodialysis (P < .001). The NSF incidence in the patients with acute renal failure who received a high dose when their creatinine level was increasing was 19% (11 of 58 patients) when hemodialysis was delayed for longer than 2 days. More patients with NSF had proinflammatory events, and compared with patients without NSF, these patients had lower pH, younger age, lower eGFR, elevated serum phosphorus levels, and a longer delay between GBCA injection and hemodialysis. For patients with an eGFR lower than 15 mL/min, hemodialysis helped to prevent NSF. For patients with an eGFR lower than 30 mL/min who received a high dose of GBCA, acute renal failure, delayed hemodialysis after contrast agent injection, proinflammatory events, and hyperphosphatemia were associated with increased risk of NSF.

Nephrogenic systemic fibrosis: epidemiology update

Purpose of review The aim of this article is to outline the history of nephrogenic systemic fibrosis, a new and serious disease of patients with renal failure, and to give an update on its aetiology and prevalence. Recent findings Epidemiological and histochemical studies demonstrated that gadolinium-containing contrast agents used for magnetic resonance imaging have an essential causative role in most, if not all, cases of nephrogenic systemic fibrosis. One particular agent, gadodiamide, caused the majority of cases, but gadopentetate dimeglumine has also been implicated in several cases. Increasingly poor renal function, aberrations in calcium–phosphate metabolism and erythropoietin treatment seem to increase the risk of the disease and its severity. Up to 25–30% of patients with renal failure exposed to gadolinium-based contrast agents may develop nephrogenic systemic disease. The figure varies with type of gadolinium-based contrast agent and patient characteristics. There is no established curative treatment of the disease, but rapid recovery of renal function or hastened kidney transplantation may be beneficial. Summary It is mandatory to avoid exposing patients with renal failure to gadodiamide and gadopentetate dimeglumine. Other linear gadolinium-based contrast agents should be used with great caution. It is premature to conclude whether cyclic gadolinium-based contrast agents are totally safe in renal failure.

Nephrogenic systemic fibrosis: epidemiology update

The aim of this article is to outline the history of nephrogenic systemic fibrosis, a new and serious disease of patients with renal failure, and to give an update on its aetiology and prevalence. Epidemiological and histochemical studies demonstrated that gadolinium-containing contrast agents used for magnetic resonance imaging have an essential causative role in most, if not all, cases of nephrogenic systemic fibrosis. One particular agent, gadodiamide, caused the majority of cases, but gadopentetate dimeglumine has also been implicated in several cases. Increasingly poor renal function, aberrations in calcium-phosphate metabolism and erythropoietin treatment seem to increase the risk of the disease and its severity. Up to 25-30% of patients with renal failure exposed to gadolinium-based contrast agents may develop nephrogenic systemic disease. The figure varies with type of gadolinium-based contrast agent and patient characteristics. There is no established curative treatment of the disease, but rapid recovery of renal function or hastened kidney transplantation may be beneficial. It is mandatory to avoid exposing patients with renal failure to gadodiamide and gadopentetate dimeglumine. Other linear gadolinium-based contrast agents should be used with great caution. It is premature to conclude whether cyclic gadolinium-based contrast agents are totally safe in renal failure.

Nephrogenic systemic fibrosis: chronic imaging findings and review of the medical literature

Nephrogenic systemic fibrosis (NSF) is a systemic fibrosing disorder which has been strongly associated with exposure to gadolinium-based contrast media (GBCM) in the setting of renal insufficiency. Although this disorder primarily affects the skin, it can result in severe joint contractures, disabilities and even death. However, to date, there have been no published studies reporting chronic imaging findings of NSF. In this report we present three biopsy-proven cases of NSF with the associated chronic MRI, radiographic and bone scintigraphy findings. Two of the patients had been exposed to gadodiamide, and one had been exposed to gadopentetate dimeglumine prior to the onset of NSF. Two are newly reported cases. One patient's subacute imaging findings have previously been reported, but significant chronic images will now be presented. This patient became severely disabled from contractures and developed long bone smooth periosteal reaction, extensive intra-articular and periarticular calcifications, musculotendinous heterotopic ossification and ankylosis of several joints. One of the patients underwent renal transplantation 6 months after GBCM exposure, with near complete resolution of the skin fibrosis. The third patient had persistent MRI findings of skin thickening, with low T1 and high T2 signal intensity 5 years after exposure to gadodiamide. A review of the medical literature is provided, emphasizing the association of NSF with various GBCM. These cases broaden our understanding of the long-term imaging findings and complications of NSF and the stratified risk of NSF with various GBCM.

Gadolinium-Based Contrast Exposure, Nephrogenic Systemic Fibrosis, and Gadolinium Detection in Tissue

The objective of our study was to retrospectively review one institution's cases of nephrogenic systemic fibrosis (NSF), evaluate possible associated factors, determine the prevalence of NSF, and search for gadolinium in skin samples obtained from patients with NSF. A retrospective review of our dermatopathology database from 1997 to 2007 was performed to search for patients with NSF. The records of patients with NSF were reviewed for factors suspected to be associated with NSF such as acidosis, low hemoglobin levels, low serum calcium levels, inflammatory conditions, serum antibodies, pharmaceutical erythropoietin, angiotensin-converting enzyme inhibitors, gadolinium-based contrast agents (GBCAs), renal failure, and dialysis. The biopsy samples from NSF patients and from control subjects were examined with energy-dispersive X-ray spectroscopy to detect gadolinium. Retrospective chart reviews of patients evaluated at our local dialysis center and our dermatology clinic were conducted to identify patients who underwent MRI, who had NSF managed exclusively by our tertiary referral centers, or both from 1997 to 2007. Seven cases of NSF were found in the dermatopathology database. Two of the seven patients were also followed up at our outpatient dialysis clinic. No other cases of NSF were discovered within the dialysis clinic's population exclusively followed within our institution. All seven dermatopathology database NSF patients developed symptoms of NSF after receiving GBCAs during renal failure and showed concomitant proinflammatory conditions. No other proposed risk factors were uniformly present in these NSF cases. All four NSF patients with chronic renal failure developed NSF after hemodialysis, with one patient dialyzed 12 hours after receiving a contrast dose. Gadodiamide was the only GBCA that all seven NSF patients received before symptom onset. Symptom onset was from 3 weeks to 18 months after GBCA exposure, with cumulative GBCA doses ranging from 0.16 to 0.43 mmol/kg. Gadolinium was detected in six of seven NSF patients' skin biopsies. Seven of eight random control specimens obtained from three healthy control subjects, three patients with renal insufficiency who had not been exposed to gadodiamide, and two patients without renal disease who had been exposed to gadodiamide were negative. Seventy-two dialysis clinic patients underwent 127 contrast-enhanced MR examinations from 1997 to 2007. Eighteen patients received gadopentetate, none of whom developed NSF. Sixty-three patients received gadodiamide, two of whom developed NSF (prevalence of NSF in patients exposed to GBCA, 2.8%; odds ratio, 0.82 [95% CI, 0.04-18.10]; likelihood ratio, 1.16 [95% CI, 1.06-1.26]). Nine patients received both contrast agents. An association with GBCAs in the development of NSF is suggested in the setting of renal insufficiency, but other factors seem to play a role. Dialysis did not prevent the development of NSF. Gadolinium was detected in skin samples from NSF patients.

Nephrogenic systemic fibrosis associated with gadolinium based contrast agents: A summary of the medical literature reporting

Nephrogenic systemic fibrosis (NSF) is a systemic fibrosing disorder that principally affects the skin, but can involve virtually any tissue in the human body and result in significant disability and even death. Since 2006 numerous retrospective case reports and case series have reported a very strong association of this disease with exposure to gadolinium-based contrast agents (Gd-CA) for MR imaging in the setting of severe or end-stage renal disease. The purpose of this report is to summarize the medical literature reporting of biopsy-proven NSF cases in which the authors specifically investigated patient exposure to Gd-CA. A Pub Med MEDLINE search was performed using the key words—nephrogenic systemic fibrosis and nephrogenic fibrosing dermopathy. All case reports and case series of NSF were reviewed to determine if patients had a preceding exposure to Gd-CA and which specific Gd-CA was involved. If the original reports did not clarify the specific Gd-CA, I reviewed follow-up letters to the editors or contacted the authors to clarify which specific Gd-CA were linked to the NSF cases. If several reports originated from the same institution, clarification was also obtained to avoid redundant reporting. As of February 1, 2008 there have been 190 biopsy-proven cases of NSF published in the peer-reviewed literature with the following associations: 157 gadodiamide (Omniscan, GE Healthcare), 8 gadopentetate (Magnevist, Bayer Healthcare), 3 gadoversetamide (OptiMARK, Covidien), and 18 unspecified Gd-CA, and 4 confounded cases with more than one Gd-CA. Five cases of NSF were unassociated with Gd-CA.

A follow-up of four cases of nephrogenic systemic fibrosis: is gadolinium the specific trigger?

We originally reported four patients with nephrogenic systemic fibrosis (NSF) in the British Journal of Dermatology in 2003, and now present an update of their outcome. All four patients (three females and one male) presented within a six month period at our institution, and had received gadolinium contrast agents prior to disease onset. Two patients with functioning renal transplants had limited cutaneous disease, two patients maintained on haemodialysis had more severe disease and died one year after disease onset. Gadolinium deposition was demonstrated in lesional skin of one patient using electron microscopy and energy dispersive spectroscopy.

An epidemic outbreak of nephrogenic systemic fibrosis in a Danish hospital

The nephrological department of Copenhagen University Hospital Herlev experienced an epidemic accumulation of patients developing nephrogenic systemic fibrosis in the period 2002–2006. Systematic studies of these patients revealed that they all had a gadodiamide-enhanced magnetic resonance examination prior to their symptoms, and that they all had severe renal insufficiency (chronic kidney disease stage 5) at the time of their exposure to gadodiamide. Besides exposure to gadodiamide, our analyses indicated that increasing cumulative gadodiamide exposure (i.e. repeated exposures), and higher serum concentrations of ionized calcium and phosphate were cofactors that raised the risk of developing nephrogenic systemic fibrosis. Higher cumulative gadodiamide exposure, higher prescribed erythropoietin dosage at exposure, and being hemodialysis patient were three factors associated with nephrogenic systemic fibrosis in its most severe form. Retrospective reviews of patients records and patient interviews revealed the large variability in symptoms and clinical course of nephrogenic systemic fibrosis, but also highlighted that the typical initial symptoms were symmetric swelling, discoloration and pain of lower legs, whereas the typical late symptoms of severely affected patients were skin thickening, stiffness, contractures, and debilitating disabilities. In conclusion, nephrogenic systemic fibrosis is a serious iatrogenic disease of patients with renal insufficiency caused by some Gd-containing contrast agents, in particular gadodiamide. Unfortunately, there is no proven curative treatment. It is therefore essential that future cases of nephrogenic systemic fibrosis are prevented.

What Causes Nephrogenic Systemic Fibrosis?

What Causes Nephrogenic Systemic Fibrosis?

Risk for Nephrogenic Systemic Fibrosis with Gadoteridol (ProHance) in Patients Who Are on Long-Term Hemodialysis

Recent studies strongly link nephrogenic systemic fibrosis to gadolinium administration for magnetic resonance imaging. In a recent advisory, the Food and Drug Administration stated that all gadolinium-containing chelates are potentially associated with nephrogenic systemic fibrosis; however, most reported cases are linked to gadodiamide (Omniscan) and gadopentetate dimeglumine (Magnevist). Given the severe consequences of nephrogenic systemic fibrosis, it is critical to define the risks associated with each gadolinium-containing chelate. The purpose of this study was to examine nephrogenic systemic fibrosis risk in a hemodialysis population exposed to gadoteridol (ProHance). Appointment logs were used to generate a database of all long-term hemodialysis patients at the Dallas Veterans Affairs hospital since August 2001. These patients were then examined in the Veterans Affair's electronic medical record system for gadolinium exposure during magnetic resonance imaging from 2000 through 2007, a period during which gadoteridol was the sole contrast agent used. A total of 141 patients were identified with 198 gadoteridol exposures. No cases of nephrogenic systemic fibrosis were identified. The observed frequency of nephrogenic systemic fibrosis was compared with the expected frequency (2.4%) using one-way chi(2) and binomial analysis, yielding a P < 0.05, indicating that the result was not explained by chance alone. It is concluded that the risk for nephrogenic systemic fibrosis with gadoteridol in patients who are on long-term hemodialysis may be lower than with gadodiamide and gadopentetate dimeglumine.

NSF: WHAT WE KNOW AND WHAT WE NEED TO KNOW: Nephrogenic Systemic Fibrosis and Gadolinium‐Based Contrast: Medico‐Legal Implications

AbstractThe epidemiologic link between exposure to gadolinium‐based contrast (GBC) agents and the development of nephrogenic systemic fibrosis (NSF) in patients with moderate to severe end‐stage renal disease (ESRD) has caused concern among physicians, particularly radiologists and nephrologists. The concerns of manufacturers of GBC agents and the U.S. Food and Drug Administration (FDA) have led to the publication of product label warnings and a Public Health Advisory. As more cases of NSF associated with GBC agent exposure have surfaced, manufacturers and physicians have become increasingly concerned about the potential for litigation. To date, litigation has been directed primarily at manufacturers of GBC agents and has focused on package warnings. Thus, physicians should stay abreast of FDA warnings, keep an open line of communication with manufacturers of GBC agents, and review package warnings.

The Impact of NSF on the Care of Patients With Kidney Disease

The association of nephrogenic systemic fibrosis (NSF) with the use of gadolinium-based magnetic resonance contrast agents (GBMCAs) has greatly affected the care of patients with kidney disease. Nephrogenic systemic fibrosis has been reported in patients with end-stage renal disease, chronic kidney disease (CKD) and acute kidney injury. The majority of cases have occurred in patients with end-stage renal disease, but cases of NSF have been reported in CKD patients with glomerular filtration rates less than 30 mL/min/1.73 m(2). Odds ratios have ranged between 8.97 and 32.5 among patients exposed to GBMCAs. Given the significant morbidity, disability, and mortality associated with NSF, it is imperative to properly and preemptively identify those patients at risk. Patients with end-stage renal disease seem to be at highest risk, particularly those maintained on peritoneal dialysis (PD). Although there are no data to support a role for hemodialysis in reducing the risk for NSF after the administration of GBMCAs, hemodialysis is recommended within 2 to 3 hours. Patients maintained on PD and those with CKD present a challenge, as they do not typically have vascular access for hemodialysis, yet the clearance of GBMCAs is very low, and it may be prudent to consider hemodialysis especially for PD patients. Gadolinium-based magnetic resonance contrast agents are removed by dialysis, with estimates that about 99% of a dose is removed after 3 to 4 sessions of hemodialysis. The elimination half-life averaged 9 hours in patients with stage 4 CKD (glomerular filtration rate <30 mL/min/1.73 m(2)) compared with 1.5 hours in those with normal glomerular filtration rates. This prolonged elimination and longer exposure may be important factors in predisposing to NSF.

Gadolinium-Containing MRI Contrast Agents: Important Variations on a Theme for NSF

Millions of doses of gadolinium-based contrast agents (GBCAs) are administered annually to improve the clinical utility of magnetic resonance imaging. All the approved agents incorporate one atom of the rare earth metal gadolinium into a chelate to improve the safety of the ordinarily toxic free gadolinium. The undeniable epidemiologic link between GBCAs and nephrogenic systemic fibrosis (NSF) has prompted renewed investigation into the different chemical properties of the GBCAs despite their clinical interchangeability. Gadolinium-based contrast agents can be divided into different categories: linear versus macrocyclic structure, ionic versus nonionic, and non-protein-binding versus protein-binding agents. The GBCAs differ significantly with respect to transmetallation and kinetic and thermodynamic stability and therefore their propensity to release free gadolinium, which is hypothesized to induce NSF. That gadodiamide, with its susceptibility to transmetallation and relatively low thermodynamic and kinetic stability, is associated with the most cases of NSF supports this hypothesis. On the other hand, the greater stability of a macrocyclic agent hypothetically would confer a greater safety margin with regard to NSF. Because few published data on an experimental model of NSF exist, continuing vigilance is necessary to report new cases of NSF, especially with regard to the agents with small market share.

Nephrogenic Systemic Fibrosis/Nephrogenic Fibrosing Dermopathy

Nephrogenic systemic fibrosis (NSF) is a rare, idiopathic systemic fibrosing disorder that is predominantly characterized by a clinicopathologically distinct symmetric dermopathy and debilitating joint contractures. The condition affects patients with renal insufficiency and appears to show improvement with prompt restoration of renal function. Although NSF was initially reported in the United States as nephrogenic fibrosing dermopathy, it has subsequently been described in patients in Europe and Asia. More than 215 cases of NSF have been reported in the nephrogenic fibrosing dermopathy/NSF registry that is currently maintained at the Yale University. Recent reports suggest a possible etiologic link between systemic administration of gadolinium-containing magnetic resonance imaging contrast agents in patients with renal insufficiency and the development of NSF.

Nephrogenic systemic fibrosis: Center case review

To retrospectively analyze nephrogenic systemic fibrosis (NSF) cases at our center, to determine prior gadolinium based contrast agent (GBCA) administration and to evaluate possible common risk factors for the development of NSF by reviewing laboratory data and concurrent medications. A total of four data bases (pathology, MRI, dialysis, and medical records) were cross-referenced for identification and evaluation of NSF patients. Medical history of NSF patients was assessed as for previous deep venous thrombosis (DVT), surgery, or infections. Laboratory data (creatinine, anion gap, calcium, phosphorus, and albumin) as well as concurrent medication were evaluated. Findings were compared to those of a control group of non-NSF dialysis patients. Between October 2003 and February 2007 a total of nine NSF cases were identified. All patients had undergone contrast-enhanced MRI prior to the diagnosis of NSF. Only one gadolinium chelate had been used at our MRI center (Omniscan, gadodiamide; GE Healthcare). Of nine patients, eight were receiving dialysis at the time of the MRI scan. During the same time 312 dialysis patients received gadodiamide. Thus, the prevalence of NSF within dialysis patients exposed to gadodiamide was 2.6%. NSF patients presented with a higher creatinine and anion gap than the control patients. Other laboratory values as well as medication did not show a significant difference. There were no patterns regarding previous history of DVT, surgery, or infection in the NSF group. Our findings are consistent with the previously reported association between gadodiamide exposure and NSF. All NSF patients had severe renal insufficiency with glomerular filtration rate (GFR) < 30 (highest GFR = 25 mL/minute) at the time of last gadodiamide administration, and on average had received 71 mL of gadodiamide over an average of 2.9 administrations.