Materiały źródłowe

• #1 Nephrogenic Systemic Fibrosis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK567754/

  Nephrogenic systemic fibrosis (NSF) is a rare yet serious fibrosing disorder primarily affecting patients with impaired renal function who have been exposed to gadolinium-based contrast agents during magnetic resonance imaging. […] Early recognition of NSF and preventive measures, particularly avoiding certain gadolinium agents in at-risk patients, is critical for reducing its incidence and associated morbidity. […] Participants learn to assess patients’ risk before imaging and recognize NSF symptoms for prompt intervention. […] The course emphasizes an interprofessional approach, promoting collaboration between radiologists, nephrologists, dermatologists, and primary care providers to ensure safe imaging practices and effective, multidisciplinary management for at-risk patients. […] Nephrogenic systemic fibrosis (NSF) is a progressive multiorgan fibrosing condition caused by exposure to gadolinium-based contrast agents (GBCAs) used for magnetic resonance imaging (MRI) in the setting of low glomerular filtration rate (GFR).

• #1 Nephrogenic Systemic Fibrosis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK567754/

  The condition was first described in 2000 when several physicians across the United States (US) noted patients with a scleroderma-type illness, and the association with GBCA administration in the setting of renal failure was solidified as more cases were presented. […] The condition was initially called nephrogenic fibrosing dermopathy due to the cutaneous manifestation. […] The American College of Radiology also has a similar classification system of 3 groups, with group 1 being the highest risk and group 3, the lowest. […] NSF is a clinicopathologic diagnosis that can be made by the history and physical and supported by a skin biopsy. […] Noting the history of GBCA exposure during the MRI examination will favor NSF among the differential diagnosis. […] Nephrogenic systemic fibrosis is considered a debilitating, progressive disease.

• #1 Nephrogenic systemic fibrosis – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/nephrogenic-systemic-fibrosis/diagnosis-treatment/drc-20352303

  Diagnosis of nephrogenic systemic fibrosis is made with: […] A sample of tissue (biopsy) taken from the skin and muscle […] Other tests as needed that may indicate involvement of muscles and internal organs.

• #1 Nephrogenic Systemic Fibrosis Workup: Laboratory Studies, Imaging Studies, Other Tests

  https://emedicine.medscape.com/article/1097889-workup

  Scleromyxedemalike skin lesions occur without associated paraproteinemia. Serum protein electrophoresis and immunoelectrophoresis results are negative. […] Nephrogenic systemic fibrosis (NSF) occurs in the setting of renal disease. Thus, abnormal values of BUN and creatinine are typically present. […] In summary, no particular laboratory abnormality is consistently linked to nephrogenic systemic fibrosis. […] No imaging study is indicated to assess nephrogenic systemic fibrosis. However, although no imaging study is indicated to assess nephrogenic systemic fibrosis, many authors have described soft tissue calcifications. […] A skin biopsy is indicated for this condition. A deep biopsy, such as an incisional biopsy or a deep punch biopsy, should be performed. A sample of dermis, subcutaneous fat, and fascia should be obtained, if possible.

• #1 Nephrogenic Systemic Fibrosis Workup: Laboratory Studies, Imaging Studies, Other Tests

  https://emedicine.medscape.com/article/1097889-workup

  In 2001, Cowper et al described the unique histopathologic features of nephrogenic systemic fibrosis, specifically, thickened collagen bundles with surrounding clefts, mucin deposition, and a proliferation of fibroblasts and elastic fibers. […] Because this condition is not commonly reported, diagnostic errors abound. Spindle cell proliferation can be confused with dermatofibrosarcoma protuberans or even spindle cell melanoma. […] Some cases of nephrogenic systemic fibrosis have manifested with a scant superficial and deep lymphocytic infiltrate, although inflammation is usually absent, and this finding should prompt consideration of an alternative diagnosis. […] Histopathologically, nephrogenic systemic fibrosis resembles scleromyxedema in that it manifests with a proliferation of dermal fibroblasts and dendritic cells, thickened collagen bundles, increased elastic fibers, and mucin deposition.

• #1

  https://link.springer.com/article/10.1007/s00467-013-2636-z

  The occurrence of NSF following gadolinium (Gd)-based contrast-agent administration was first reported in 2006. […] NSF has been reported in approximately 500 patients worldwide, but in dialysis patients given high doses of Gd, the incidence is as high as 18 %. […] The clinical picture in NSF is very similar to scleromyxedema, but NSF favors the extremities and trunk rather than the head and neck. […] Deep-skin biopsy that includes the fascia is essential for diagnosis. […] The International Center for Nephrogenic Systemic Fibrosis Research (ICNSFR) is a collaborative research group based at Yale University, New Haven, CT, USA. […] In 2009 the organization proposed a clinicopathological scoring system derived from published literature and registry data aimed at achieving diagnostic standardization.

• #1 CT and MR Imaging of Progressive Dural Involvement by Nephrogenic Systemic Fibrosis | American Journal of Neuroradiology

  http://www.ajnr.org/content/29/10/1880

  We present a patient with progressive dural calcifications, thickening, and enhancement presumably related to the development of nephrogenic systemic fibrosis (NSF). […] Nephrogenic systemic fibrosis (NSF), previously referred to as nephrogenic fibrosing dermopathy, is a relatively new, rare systemic condition first described in the literature in 2000. […] The diagnosis of NSF is made by a combination of clinical, laboratory, and histopathologic findings. […] We suggest that dural calcification, thickening, and enhancement in patients with renal disease and a history of exposure to gadolinium may represent an early manifestation of NSF.

• #1 Nephrogenic systemic fibrosis – Wikipedia

  https://en.wikipedia.org/wiki/Nephrogenic_systemic_fibrosis

  There is no specific imaging finding for NSF, and the diagnosis is a clinicopathological one, based on presentation and histological findings. […] At the microscopic level, NSF shows a proliferation of dermal fibroblasts and dendritic cells, thickened collagen bundles, increased elastic fibers, and deposits of mucin. […] The differential diagnoses for NSF include diffuse cutaneous or limited cutaneous systemic sclerosis, scleromyxedema, lipodermatosclerosis, scleroedema diabeticorum, graft versus host disease, eosinophilic fasciitis; eosinophilia-myalgia syndrome; porphyria cutanea tarda, and other disorders. The nearly universal absence of facial skin involvement in NSF, presence of yellow plaques on the sclera of the eyes, absence of Raynaud’s phenomenon, and other differences in presentation can aid the proper diagnosis. History of exposure to GBCAs would favor NSF as the differential diagnosis.

• #1 Nephrogenic Systemic Fibrosis in Patients with Chronic Kidney Disease after the Use of Gadolinium-Based Contrast Agents: A Review for the Cardiovascular Imager

  https://www.mdpi.com/2075-4418/12/8/1816

  NSF is a multisystemic fibrotic disease that affects the skin, muscle, and other organs (including lung, esophagus, and kidney) described in patients with severe renal impairment exposed to a GBCA. […] The pathophysiology and molecular mechanism of NSF are still a matter of debate. […] It is believed that the intravenous administration of some GBCAs causes a limited chelate instability that plays an essential role in the release of free Gd. […] Most GBCAs have preferential renal elimination, and their clearance is highly dependent on the glomerular filtration rate. […] The main risk factor for NSF is the presence of severe acute or chronic renal insufficiency (estimated glomerular filtration rate (eGFR): 30 mL/min/1.73 m²) or acute renal insufficiency of any severity due to hepato-renal syndrome or in the perioperative period after liver transplantation.

• #1

  https://link.springer.com/article/10.1007/s00467-013-2636-z

  The conclusion drawn in the European and American guidelines is that patients most at risk of NSF are those with CKD stage 4 or 5, including those needing dialysis and those with reduced renal function who have or are awaiting liver transplantation. […] In 2006, the Danish Medicines Agency reported 25 cases of NSF in Europe among patients with recent gadodiamide exposure. […] The approval status of commonly available Gd preparations in different jurisdictions is summarized. […] The 2010 FDA guidelines contraindicate high-risk Gd contrast agents in patients with AKI or CKD stage 45. […] Taking sales figures as a surrogate for quantities of Gd prescribed, there has been a decrease in worldwide sales of gadodiamide (the agent most frequently associated with NSF) since 2007 against a backdrop of increasing sales prior to this period.

• #1 Nephrogenic systemic fibrosis | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/nephrogenic-systemic-fibrosis?lang=us

  Nephrogenic systemic fibrosis (NSF), also known as nephrogenic fibrosing dermopathy, occurs almost exclusively in patients with renal impairment and is associated with the administration of gadolinium-based contrast agents (GBCAs) used in MRI. […] Patients should be screened for the possible risk of developing nephrogenic systemic fibrosis (NSF) by using institutional screening questionnaires and calculating the eGFR. […] New consensus suggests that kidney function screening is optional for the use of group II GBCAs. […] Recommendations pertaining to the administration of gadolinium-based contrast agents (GBCAs) in patients with kidney disease were updated in 2021, specifically with regard to the three different groups of GBCAs. […] The 2021 ACR Committee on Drugs and Contrast Media considers the risk of NSF among patients administered standard doses of group II GBCAs „sufficiently low or possibly nonexistent such that assessment of renal function with a questionnaire or laboratory testing is optional prior to intravenous administration.”

• #1 Gadolinium-containing contrast agents: new advice to minimise the risk of nephrogenic systemic fibrosis – GOV.UK

  https://www.gov.uk/drug-safety-update/gadolinium-containing-contrast-agents-new-advice-to-minimise-the-risk-of-nephrogenic-systemic-fibrosis

  The following risk-minimisation measures should be used for gadolinium-containing contrast agents for: renal-function monitoring – this should be tested in all patients receiving high-risk agents, and is generally advisable for patients receiving medium-risk or low-risk agents. […] For patients with severe renal impairment (glomerular filtration rate [GFR] 30 mL/min/173m2), use of a high-risk agent is contraindicated. […] Use of a high-risk agent is contraindicated. […] Use of any gadolinium-containing contrast agent is not recommended unless absolutely necessary.

• #1

  https://link.springer.com/article/10.1007/s00467-013-2636-z

  More than 500 cases of NSF have been reported to MedWatch, the US Food and Drugs Administration (FDA) database. […] 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. […] 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. […] The incidence of NSF following Gd administration in renally impaired patients varies between 0 % and 18 %. […] In summary, for approximately 90 % of cases, there is a clear temporal sequence between Gd administration and NSF development. […] Data show a strong association between Gd exposure and NSF, and there is a likely doseresponse relationship. […] The validity of adult data extrapolated to children is unknown.

• #1

  https://journals.lww.com/investigativeradiology/fulltext/2018/09000/10_years_of_nephrogenic_systemic_fibrosis__a.5.aspx

  The aim of this study was to critically assess the evaluation and categorization process for nephrogenic systemic fibrosis (NSF) based on reports received by Bayer from 2006 to 2016. […] A total of 779 NSF reports received by Bayer globally from 2006 to 2016 were included in the analysis. […] Bayer pharmacovigilance personnel used the information obtained from biopsy reports, detailed history of GBCA administration, medical history, and detailed history of the emergence of clinical signs and symptoms, to determine whether the case met the established Cowper/Girardi clinico-histopathological criteria for a diagnosis of NSF (Cowper/Girardi positive reports). […] Although Broome et al suggested that most NSF occurs within 3 months of the last GBCA administration, Bayer conservatively considered an 18-month onset latency as positive for a temporal association.

• #1

  https://link.springer.com/article/10.1007/s00467-013-2636-z

  Since 2007, there has been a dramatic drop in the number of NSF cases reported, with multiple large studies in Sweden and the USA identifying reduced incidence or no new cases of NSF following restrictive prescribing policies. […] Despite a myriad of other treatment options that have been proposed, the evidence is anecdotal and comes from case series and small, uncontrolled trials. […] No single treatment has convincingly shown consistent benefit. […] Current guidelines advise against administration of high-risk Gd agents to neonates and advise caution in infants. […] There is insufficient data to determine specific risk factors for NSF in children. […] No diagnoses of NSF have ever been made in neonates or infants despite thousands of Gd-enhanced MRI examinations performed. […] It remains that age-appropriate GFR levels at which it is safe to administer Gd have yet to be determined.

• #1 Nephrogenic Systemic Fibrosis: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1097889-overview

  Nephrogenic systemic fibrosis is an uncommon condition. […] The incidence of nephrogenic systemic fibrosis was 4.3 cases per 1000 patient-years. Each radiologic study using gadolinium presented a 2.4% risk for developing nephrogenic systemic fibrosis. […] Nephrogenic systemic fibrosis appears linked to increased morbidity and mortality. […] In most patients, nephrogenic systemic fibrosis is a progressive condition. […] Patients need to understand that nephrogenic systemic fibrosis in not a life-threatening disease and that it lacks effective treatment.

• #1 Nephrogenic Systemic Fibrosis | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/25703

  When the high-risk group 1 GBCAs were used, the estimated incidence was 36.5 cases per 100,000 gadolinium-enhanced MRI examinations. […] Systematic reviews have found that no patients developed biopsy-proven NSF among patients receiving group 2 or 3 GBCAs; the estimated risk of NSF when using group 2 or 3 GBCAs is less than 0.07%. […] NSF is a clinicopathologic diagnosis that can be made by the history and physical and supported by a skin biopsy. […] Noting the history of GBCA exposure during the MRI examination will favor NSF among the differential diagnosis. […] Nephrogenic systemic fibrosis is considered a debilitating, progressive disease. […] The high mortality rate does not come from the cutaneous lesions but rather from visceral fibrosis, particularly in the cardiac and respiratory systems. […] Nephrogenic systemic fibrosis complicates the already existing kidney disease.

• #1 Nephrogenic Systemic Fibrosis in Denmark– A Nationwide Investigation | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0082037

  Nephrogenic systemic fibrosis is a debilitating and painful disorder with an increased stimulation of the connective tissue in the skin and systemic tissues. The disease is associated with exposure to gadolinium-based contrast agent used in magnetic resonance imaging in patients with renal impairment. […] The aim of this paper is to communicate the main findings of this quest. […] The nationwide investigation in Denmark revealed the highest prevalence of NSF worldwide. This may be rooted in a high level of awareness of NSF both among doctors, politicians and, not least, the media, combined with the fact that a nationwide NSF investigation was initiated. […] In case of suspected NSF, the patient was referred to a doctor experienced in NSF who undertook a clinical examination. […] The clinical examination included a thorough evaluation of the skin.

• #1 Nephrogenic Systemic Fibrosis – What You Need to Know

  https://www.drugs.com/cg/nephrogenic-systemic-fibrosis.html

  How is NSF diagnosed? […] Blood tests may be needed to check your kidney function. […] A skin biopsy is when a sample of your skin is taken and sent to a lab for tests.

• #1 Nephrogenic Systemic Fibrosis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK567754/

  Although skin changes associated with NSF may be improved after the restoration of kidney function, especially after recovery from acute kidney injury, this condition does not usually regress spontaneously and almost always progresses relentlessly. […] The diagnosis and management of NSF need a multidisciplinary team. […] Since this disease is caused by GBCA exposure in patients with severe renal impairment, optimization of renal function is pivotal. […] The incidence rate of NSF has been drastically reduced since regulatory authorities like the FDA, American College of Radiology, and the European Society of Urogenital Radiology implemented precautionary guidelines advising radiologists to avoid high-risk GBCAs if the patient has severe renal insufficiency.

• #1 Minimizing Risk of Nephrogenic systemic fibrosis in Cardiovascular Magnetic Resonance | Journal of Cardiovascular Magnetic Resonance | Full Text

  https://jcmr-online.biomedcentral.com/articles/10.1186/1532-429X-14-31

  The vast majority of NSF cases have been reported in patients who underwent GBCA enhanced imaging however, about 5% of NSF cases showed no traceable exposure to GBCAs prior to NSF onset. […] The American NSF registry has documented over 355 proven cases of NSF so far, however, other groups have reported deviant numbers, e.g. Zou et al. report of 408 cases that were biopsy confirmed. […] Many NSF patients have improved or even been cured with restoration of normal renal function. […] The treatment options for NSF are based on case studies with limited numbers of patients. […] Considering the small numbers of case reports and the fact that NSF can possibly be avoided by preventive measures, the focus of further attention should be primarily on prevention. […] The FDA was the first agency to release a public health advisory back in 2006 when the association of GBCA and NSF was not as convincing.

• #1 Nephrogenic Systemic Fibrosis in Patients with Chronic Kidney Disease after the Use of Gadolinium-Based Contrast Agents: A Review for the Cardiovascular Imager

  https://www.mdpi.com/2075-4418/12/8/1816

  Even though the newer macrocyclic agents have proven to be much safer in patients with chronic kidney disease and end-stage renal failure, clinicians must fully understand the clinical characteristics and risk factors of this devastating pathology and maintain a high degree of suspicion to prevent and recognize it.

• #1 Nephrogenic Systemic Fibrosis in Patients with Chronic Kidney Disease after the Use of Gadolinium-Based Contrast Agents: A Review for the Cardiovascular Imager

  https://www.mdpi.com/2075-4418/12/8/1816

  A high degree of suspicion should be maintained to detect and link these symptoms with a former GBCA exposure. […] Calculation of the eGFR is vital for NSF diagnosis, as some degree of kidney dysfunction should be present in order to fulfill diagnostic criteria. […] Histopathologic examination is essential in the definitive diagnosis of NSF. […] The presence of dermal hypercellularity, CD34+ cells, procollagen type I, thick and thin collagen bundles, and osseous metaplasia significantly point toward NSF diagnosis. […] There is not a specific prophylaxis regimen to prevent the onset of NSF. […] The current approach is based on minimizing the impact of predisposing risk factors and performing hemodialysis sessions right after GBCA exposure in patients with a history of ESRD on RRT.

• #1 Nephrogenic systemic fibrosis: Clinicopathological definition and workup recommendations – EM consulte

  https://www.em-consulte.com/es/article/670866/nephrogenic-systemic-fibrosis-clinicopathological-

  A consensus scoring system incorporating a clinical and histopathological atlas was devised to guide and standardize the evaluation and diagnosis of NSF. […] […] There is no laboratory test that can be used as a gold standard to diagnose NSF. To overcome this, we relied on classic clinicopathological presentations, published sources, and consensus clinical expertise to ensure the integrity of the study population. […] […] The clinicopathological definition of NSF provides guidance to physicians for the evaluation and diagnosis of NSF. Clinical, laboratory, and histopathological features comprise a schema that excludes conditions mimicking NSF while facilitating its reproducible and accurate diagnosis, even among physicians with little prior clinical experience with this entity. This definition can serve as a working diagnostic standard for future research and as the basis for adjudicating borderline cases.

• #1

  https://journals.lww.com/investigativeradiology/fulltext/2018/09000/10_years_of_nephrogenic_systemic_fibrosis__a.5.aspx

  It is important to note that this methodology developed and implemented by Bayer is not necessarily the industry standard; there might be legitimate but slightly different processes or criteria applied by other institutions or companies. […] The goal of this analysis was to incorporate less widely discussed aspects for NSF risk assessment, such as (1) the year of market introduction and the market share of the GBCAs, (2) the interpretation of multiagent reports with special focus on the sequence of previous administration of other GBCAs, and (3) potential differences in evaluation and reporting standards by different companies. […] The second and probably most significant criticism with the current reporting standard is the limitation to single-agent cases. Nephrogenic systemic fibrosis occurs almost exclusively in multimorbid patients who received multiple doses of GBCAs spread over numerous magnetic resonance imaging (MRI) examinations.

• #1

  https://link.springer.com/article/10.1007/s00467-013-2636-z

  The unwanted effect of restricting the use of contrast-enhanced MRI may be an increase in the use of computed tomography (CT) and thereby an increase in the exposure of children to ionizing radiation. […] Although only confirmed in around 400 patients worldwide, NSF carries considerable morbidity and in rare cases may be fatal. […] There is a consistent body of evidence showing a significantly increased risk of NSF following Gd-based contrast-agent administration. […] Following the introduction of international guidelines limiting the prescription of Gd-based contrast agents and classifying individual agents as high, medium, or low risk, there has been reduced incidence of NSF.

• #2 Nephrogenic systemic fibrosis: pathogenesis, diagnosis, and therapy – PubMed

  https://pubmed.ncbi.nlm.nih.gov/19406336/

  Nephrogenic systemic fibrosis (NSF) is a newly recognized disorder occurring exclusively in patients with renal failure. Exposure to gadolinium-based magnetic resonance (MR) contrast media has been associated with subsequent development of NSF. Skin biopsy is important for confirmation of the diagnosis. The main therapeutic goal is restoration of renal function. To reduce the risk of NSF, renal function must be determined before exposure to gadolinium-containing MR contrast agents. Gadolinium-based MR contrast media should be avoided in the presence of advanced renal failure with estimated glomerular filtration rate below 30 ml/min/1.73 m2, unless the diagnostic information is essential and not available with noncontrast magnetic resonance imaging techniques. The recommended dose of contrast agent should not be exceeded. In addition, a sufficient period of time for elimination of the contrast agent from the body should be allowed before readministration of the contrast agent.

• #2 Gadolinium-Associated Nephrogenic Systemic Fibrosis | AAFP

  https://www.aafp.org/pubs/afp/issues/2009/1001/p711.html

  Nephrogenic systemic fibrosis is a progressive, potentially fatal multiorgan system fibrosing disease related to exposure of patients with renal failure to the gadolinium-based contrast agents used in magnetic resonance imaging. […] The diagnosis of nephrogenic systemic fibrosis is made by high clinical suspicion in at-risk patients and confirmed by the characteristic findings on skin biopsy. It is recommended that the biopsy extend into the subcutaneous fat or fascia by incisional or deep punch biopsies. […] The pathophysiology behind nephrogenic systemic fibrosis is unclear. […] Currently, there is no effective treatment for nephrogenic systemic fibrosis. […] Prevention of nephrogenic systemic fibrosis is the best practice. […] Nephrogenic systemic fibrosis is an important disease of which all physicians who order MRI with gadolinium-based contrast agents must be aware.

• #2 Mayo Clinic Health Library – Nephrogenic systemic fibrosis | Swiss Medical Network

  https://www.swissmedical.net/fr/healtcare-library/con-20166959

  Nephrogenic systemic fibrosis is a rare disease that occurs mainly in people with advanced kidney failure with or without dialysis. […] Diagnosis of nephrogenic systemic fibrosis is made with: […] Physical exam for signs and symptoms of the disease, and evaluation for a possible history of MRI using a gadolinium-based contrast agent when advanced kidney disease is present. […] A sample of tissue (biopsy) taken from the skin and muscle. […] Other tests as needed that may indicate involvement of muscles and internal organs.

• #2 Nephrogenic Systemic Fibrosis (NSF): Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17783-nephrogenic-systemic-fibrosis-nsf

  Nephrogenic systemic fibrosis (NSF) is a rare condition that affects your skin and organs. It occurs in people with kidney disease after exposure to gadolinium, which is used in certain imaging tests. […] Nephrogenic systemic fibrosis (NSF) is a rare disease that occurs mostly in people with kidney disease after exposure to gadolinium-based contrast agents (GBCAs). […] The U.S. Food Drug Administration (FDA) requires warning labels on certain GBCAs that say the product shouldnt be given to people with kidney disease because its a known cause of NSF. […] There havent been any new reports of NSF in almost 15 years. This is due to the development and use of more stable, less toxic GBCAs. […] The only way to be certain you have NSF is to have a skin biopsy. During a skin biopsy, a healthcare provider removes a sample of your skin and sends it to a lab for analysis.

• #2 Nephrogenic systemic fibrosis – Wikipedia

  https://en.wikipedia.org/wiki/Nephrogenic_systemic_fibrosis

  There is no specific imaging finding for NSF, and the diagnosis is a clinicopathological one, based on presentation and histological findings. […] At the microscopic level, NSF shows a proliferation of dermal fibroblasts and dendritic cells, thickened collagen bundles, increased elastic fibers, and deposits of mucin. […] The differential diagnoses for NSF include diffuse cutaneous or limited cutaneous systemic sclerosis, scleromyxedema, lipodermatosclerosis, scleroedema diabeticorum, graft versus host disease, eosinophilic fasciitis; eosinophilia-myalgia syndrome; porphyria cutanea tarda, and other disorders. The nearly universal absence of facial skin involvement in NSF, presence of yellow plaques on the sclera of the eyes, absence of Raynaud’s phenomenon, and other differences in presentation can aid the proper diagnosis. History of exposure to GBCAs would favor NSF as the differential diagnosis.

• #2 Nephrogenic systemic fibrosis: Clinicopathological definition and workup recommendations – EM consulte

  https://www.em-consulte.com/es/article/670866/nephrogenic-systemic-fibrosis-clinicopathological-

  A consensus scoring system incorporating a clinical and histopathological atlas was devised to guide and standardize the evaluation and diagnosis of NSF. […] […] There is no laboratory test that can be used as a gold standard to diagnose NSF. To overcome this, we relied on classic clinicopathological presentations, published sources, and consensus clinical expertise to ensure the integrity of the study population. […] […] The clinicopathological definition of NSF provides guidance to physicians for the evaluation and diagnosis of NSF. Clinical, laboratory, and histopathological features comprise a schema that excludes conditions mimicking NSF while facilitating its reproducible and accurate diagnosis, even among physicians with little prior clinical experience with this entity. This definition can serve as a working diagnostic standard for future research and as the basis for adjudicating borderline cases.

• #2 Nephrogenic Systemic Fibrosis – MD Searchlight

  https://mdsearchlight.com/medications/nephrogenic-systemic-fibrosis/

  The types of tests that may be needed to diagnose Nephrogenic Systemic Fibrosis (NSF) include: Medical history evaluation, Physical examination, eGFR kidney function test, Tissue sample (deep skin biopsy), Blood tests. […] Nephrogenic Systemic Fibrosis can develop in connection with the use of certain types of substances known as GBCAs, especially when the kidneys are not functioning properly. […] The prognosis for Nephrogenic Systemic Fibrosis (NSF) depends on how much the fibrosis has affected the internal organs. […] Nephrogenic Systemic Fibrosis (NSF) is currently incurable, but there are various treatment methods that can help alleviate symptoms. […] Restoring kidney function is a crucial first step in slowing down or halting the progression of the disease.

• #2 SciELO Brazil – Nephrogenic systemic fibrosis: concepts and perspectives Nephrogenic systemic fibrosis: concepts and perspectives

  https://www.scielo.br/j/abd/a/YRrkNwbg8vdYmP5SR85wmcG/?lang=en

  Nephrogenic systemic fibrosis is a chronic, progressive condition that develops in some patients with renal impairment after exposure to gadolinium-based contrast agents used in magnetic resonance imaging. […] The diagnosis of the disease requires careful clinicopathological correlation. […] The diagnosis must be established by a correlation between clinical, laboratory and histopathological findings. The presence of fibrotic skin changes in a patient with renal impairment should prompt further investigation. […] The diagnosis is confirmed by anatomopathological examination and the findings vary according to the stage of evolution of the disease. […] There are no laboratory and pathognomonic histopathological findings or findings consistently associated with the disease that allow its diagnosis in isolation.

• #2 Enhanced MRI and the Nephrogenic Systemic Fibrosis Debate

  https://www.medscape.org/viewarticle/708576

  In 2006 and 2007, Grobner and Marckman linked the development of NSF to the use of gadolinium-based MRI contrast agents. […] The appearance of NSF coincides with the timeline of when physicians started using high-dose gadolinium MRA in patients with renal problems. […] The exact mechanism of triggering of NSF is not well understood to date. […] The only way to make this diagnosis is by deep skin biopsy. […] There is no role for diagnostic imaging in the diagnosis of NSF. […] NSF can potentially mimic other disorders, including scleromyxedema, systemic sclerosis, pretibial myxedema, and eosinophilic fasciitis. […] Improving renal function can potentially slow down and in some cases, reverse the course of the disease. […] The natural history of NSF is not well understood. […] In summary, NSF has not occurred in any patient with a GFR of greater than 30 mL/min/1.73 m2. […] Although certain gadolinium compounds have been implicated more often than others, there is no convincing evidence that others are completely safe, and therefore we must exercise caution with the use of all gadolinium agents until more research is completed.

• #2 Erythema nodosum masking nephrogenic systemic fibrosis as initial skin manifestation | BMC Nephrology | Full Text

  https://bmcnephrol.biomedcentral.com/articles/10.1186/s12882-017-0666-7

  The predominant symptom at the later stage was symmetrical skin stiffness of the extremities with or without restricted joint motion. […] After exposure to GBCAs, the time of onset of NSF ranges from a few days to 6 months. […] In our case, the possible differentials for thickening and hardening of the skin overlying the limbs and trunk involved a variety of other disorders, such as systemic sclerosis (scleroderma), scleromyxedema, eosinophilic fasciitis and calciphylaxis. […] Unfortunately, there is no effective treatment available to treat NSF. […] We hope by highlighting this case, we can avoid the potential pitfall of missing early phase of NSF in CKD patients who received CBGA.

• #2 SciELO Brazil – Nephrogenic systemic fibrosis: concepts and perspectives Nephrogenic systemic fibrosis: concepts and perspectives

  https://www.scielo.br/j/abd/a/YRrkNwbg8vdYmP5SR85wmcG/?lang=en

  The presence of Gd deposits can be seen via spectrometry, but their intensity does not correlate with the extent of clinical involvement. […] Girardi et al. proposed major and minor clinical and histopathological criteria for the diagnosis of NSF. […] The precise role of fibrocytes in the etiopathogenesis of the disease is unclear. It is not known whether they trigger the fibrotic process (due to their ability to migrate to and enter damaged tissues) or simply maintain the development of fibrotic lesions. […] The differential diagnosis of NSF includes other disorders involving the presence of skin fibrosis and is done through clinical and histopathological findings. […] Exclusive involvement of patients with renal impairment, history of exposure to GBCA and typical distribution of skin lesions of NSF favor the differential diagnosis.

• #2 Contrast Agent Safety and Nephrogenic Systemic Fibrosis | Radiology Key

  https://radiologykey.com/contrast-agent-safety-and-nephrogenic-systemic-fibrosis/

  There is a strong connection between NSF and poor renal function. […] One study looking at NSF incidence among patients receiving gadolinium contrast at two large medical centers in the United States found that all patients developing NSF had an estimated glomerular filtration rate (GFR) less than 30 mL/min/1.73 m2. […] No patient developed NSF after receiving standard-dose gadolinium contrast. […] The study concluded that acute renal failure and delayed hemodialysis were NSF risk factors for patients with a GFR less than 30 mL/min/1.73 m2 who received high-dose gadolinium. […] In addition to renal function, the type (structure) and dose of gadolinium contrast administered are other possible risk factors for NSF. […] Higher doses of gadolinium contrast appear to be a risk factor for NSF when compared to the standard dose of 0.1 mmol/kg.

• #2 Minimizing Risk of Nephrogenic systemic fibrosis in Cardiovascular Magnetic Resonance | Journal of Cardiovascular Magnetic Resonance | Full Text

  https://jcmr-online.biomedcentral.com/articles/10.1186/1532-429X-14-31

  The European Medicines Agency (EMA) has defined three risk classes of Gadolinium- based contrast agents. […] The EMEAs recommendations on the use of GBCAs are based on this classification. […] The American College of Radiology (ACR) recommendations of 2010 agree with the other guidelines on the associations of GBCA exposure, dosage relations and kidney function.

• #2 Nephrogenic systemic fibrosis | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/nephrogenic-systemic-fibrosis?lang=us

  Group II GBCAs may be administered to high-risk patients without kidney function screening and without contact with the referring provider, depending on individual practice patterns. […] The risk estimate of NSF for group II GBCAs in patients with stage 5 chronic kidney disease is based on data from 2581 individuals. It is possible that NSF may rarely occur in this population.

• #2 Nephrogenic Systemic Fibrosis in Patients with Chronic Kidney Disease after the Use of Gadolinium-Based Contrast Agents: A Review for the Cardiovascular Imager

  https://www.mdpi.com/2075-4418/12/8/1816

  A high degree of suspicion should be maintained to detect and link these symptoms with a former GBCA exposure. […] Calculation of the eGFR is vital for NSF diagnosis, as some degree of kidney dysfunction should be present in order to fulfill diagnostic criteria. […] Histopathologic examination is essential in the definitive diagnosis of NSF. […] The presence of dermal hypercellularity, CD34+ cells, procollagen type I, thick and thin collagen bundles, and osseous metaplasia significantly point toward NSF diagnosis. […] There is not a specific prophylaxis regimen to prevent the onset of NSF. […] The current approach is based on minimizing the impact of predisposing risk factors and performing hemodialysis sessions right after GBCA exposure in patients with a history of ESRD on RRT.

• #2 Potential Side Effects of Gadolinium-Enhanced MRI | AAFP

  https://www.aafp.org/pubs/afp/issues/2009/0801/p214.html

  The diagnosis of NSF is relatively new, with the first cases being reported in 1997. NSF is a fibrosing disorder in patients with chronic kidney disease that has been linked to the use of gadolinium for enhanced MRI. […] More than 95 percent of the cases reported to the International NSF Registry involved patients who had been exposed to gadolinium within two to three months of disease onset. […] The U.S. Food and Drug Administration recommends using gadolinium only when clearly necessary in patients with stage 4 and 5 renal disease, which it defines as a glomerular filtration rate lower than 30 mL per minute per 1.73 m2. […] If patients with renal insufficiency receive gadolinium, then hemodialysis should be promptly started after the imaging study is completed. […] Treatments for NSF are limited but include renal transplantation, extracorporeal photopheresis, ultraviolet A phototherapy, and plasmapheresis.

• #2

  https://link.springer.com/article/10.1007/s00467-013-2636-z

  More than 500 cases of NSF have been reported to MedWatch, the US Food and Drugs Administration (FDA) database. […] 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. […] 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. […] The incidence of NSF following Gd administration in renally impaired patients varies between 0 % and 18 %. […] In summary, for approximately 90 % of cases, there is a clear temporal sequence between Gd administration and NSF development. […] Data show a strong association between Gd exposure and NSF, and there is a likely doseresponse relationship. […] The validity of adult data extrapolated to children is unknown.

• #2 Contrast Agent Safety and Nephrogenic Systemic Fibrosis | Radiology Key

  https://radiologykey.com/contrast-agent-safety-and-nephrogenic-systemic-fibrosis/

  Determining the estimated GFR as an indicator of kidney dysfunction is essential in determining who is at risk for NSF. […] A full-body skin examination should be performed in patients with suspected NSF by a dermatologist, rheumatologist, or other health care professional who is familiar with clinical features of NSF and how to distinguish them from other disorders that are in the differential diagnosis of NSF. […] Laboratory tests (no lab test is diagnostic for NSF) GFR for monitoring renal function in NSF progression/remission OR if gadolinium contrast is to be administered to a patient at risk for NSF. […] There are multiple other conditions with prominent dermatologic findings that are in the differential diagnosis of NSF.

• #2 Nephrogenic systemic fibrosis/nephrogenic fibrosing dermopathy in advanced kidney disease – UpToDate

  https://www.uptodate.com/contents/nephrogenic-systemic-fibrosis-nephrogenic-fibrosing-dermopathy-in-advanced-kidney-disease

  Nephrogenic systemic fibrosis (NSF) is a disorder seen only in patients with advanced kidney disease and is characterized by two primary features: […] NSF was originally named nephrogenic fibrosing dermopathy (NFD) because of the characteristic skin findings. However, subsequent studies showed that some patients had fibrosis of deeper structures, including muscle, fascia, lungs, and heart. […] While NSF is only seen among patients with kidney disease, retention of gadolinium (Gd) has been demonstrated in other organs (eg, brain) in patients without and with kidney disease.

• #2 Erythema nodosum masking nephrogenic systemic fibrosis as initial skin manifestation | BMC Nephrology | Full Text

  https://bmcnephrol.biomedcentral.com/articles/10.1186/s12882-017-0666-7

  Nephrogenic systemic fibrosis (NSF) is a complication of the gadolinium-based contrast agent used in imaging studies. It is typically characterised by hard, erythematous and indurated skin plaques with surrounding subcutaneous oedema. […] A repeated skin biopsy done three months later confirmed the diagnosis of NSF. […] The appearance of EN-like disease can be one of the early manifestations of NSF. We hope that early recognition of this unusual presentation can alert the physician or nephrologist to the potential diagnosis of NSF. […] In the majority of NSF patients, symptomatic skin involvement is the initial presentation of the disease. […] The common link to all patients developing NSF is the history of renal impairment and gadolinium-based contrast agent (GBCA) exposure. […] The risk of developing NSF in a patient with severe kidney impairment following exposure to GBCA is roughly 4% regardless of gender, race or age.

• #2 Nephrogenic Systemic Fibrosis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK567754/

  Although skin changes associated with NSF may be improved after the restoration of kidney function, especially after recovery from acute kidney injury, this condition does not usually regress spontaneously and almost always progresses relentlessly. […] The diagnosis and management of NSF need a multidisciplinary team. […] Since this disease is caused by GBCA exposure in patients with severe renal impairment, optimization of renal function is pivotal. […] The incidence rate of NSF has been drastically reduced since regulatory authorities like the FDA, American College of Radiology, and the European Society of Urogenital Radiology implemented precautionary guidelines advising radiologists to avoid high-risk GBCAs if the patient has severe renal insufficiency.

• #2 Nephrogenic Systemic Fibrosis (NSF): Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17783-nephrogenic-systemic-fibrosis-nsf

  Currently, there are no approved treatments for NSF. […] Avoiding older GBCAs in people with kidney disease is the best way to prevent NSF. […] The FDA recommends against using gadolinium-based contrast agents in people with certain kidney conditions unless there are no other options. […] With preventive measures in place, youre unlikely to get a diagnosis of NSF. But if you have kidney disease and require an MRI, talk to your healthcare provider about NSF and your risk.

• #2 Nephrogenic Systemic Fibrosis: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1097889-overview

  The pathophysiology of nephrogenic systemic fibrosis is related to the exposure of patients with renal insufficiency to gadolinium in association with imaging studies. […] The safety of gadopentetate linear product might be no different from macrocyclic preparations such as gadodiamide (Omniscan), but guidelines should be followed in all gadolinium products. […] The cause of nephrogenic systemic fibrosis is the connexation of renal insufficiency and gadolinium exposure from imaging studies. […] Gadolinium-based contrast agents (Ablavar, Eovist, Magnevist, MultiHance, Omniscan, OptiMARK, ProHance) have recently been linked to the development of nephrogenic systemic fibrosis. […] On September 9, 2010, the FDA announced it is requiring that gadolinium-based contrast agents (GBCAs) carry new warnings on their labels about the risk for nephrogenic systemic fibrosis and its association when administered to certain patients with kidney disease.

• #2

  https://link.springer.com/article/10.1007/s00467-013-2636-z

  The unwanted effect of restricting the use of contrast-enhanced MRI may be an increase in the use of computed tomography (CT) and thereby an increase in the exposure of children to ionizing radiation. […] Although only confirmed in around 400 patients worldwide, NSF carries considerable morbidity and in rare cases may be fatal. […] There is a consistent body of evidence showing a significantly increased risk of NSF following Gd-based contrast-agent administration. […] Following the introduction of international guidelines limiting the prescription of Gd-based contrast agents and classifying individual agents as high, medium, or low risk, there has been reduced incidence of NSF.

• #2 Nephrogenic Systemic Fibrosis in Denmark– A Nationwide Investigation | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0082037

  The patient was diagnosed with NSF if clinical and pathological observations were consistent with the established diagnostic criteria. […] The present nationwide investigation was challenged by several factors. […] The rise in the number of reported NSF cases and subsequent pressure from the media made a nationwide investigation mandatory in Denmark. […] The outcome of the nationwide investigation revealed 65 NSF patients in a population of 5.6 million inhabitants in Denmark, or 12 per million. This high prevalence of NSF worldwide may be rooted in a high level of awareness of NSF both among Danish doctors, politicians and, not least, the media, combined with the fact that Denmark initiated the first nationwide NSF investigation.

• #3 Nephrogenic systemic fibrosis – Wikipedia

  https://en.wikipedia.org/wiki/Nephrogenic_systemic_fibrosis

  There is no specific imaging finding for NSF, and the diagnosis is a clinicopathological one, based on presentation and histological findings. […] At the microscopic level, NSF shows a proliferation of dermal fibroblasts and dendritic cells, thickened collagen bundles, increased elastic fibers, and deposits of mucin. […] The differential diagnoses for NSF include diffuse cutaneous or limited cutaneous systemic sclerosis, scleromyxedema, lipodermatosclerosis, scleroedema diabeticorum, graft versus host disease, eosinophilic fasciitis; eosinophilia-myalgia syndrome; porphyria cutanea tarda, and other disorders. The nearly universal absence of facial skin involvement in NSF, presence of yellow plaques on the sclera of the eyes, absence of Raynaud’s phenomenon, and other differences in presentation can aid the proper diagnosis. History of exposure to GBCAs would favor NSF as the differential diagnosis.

• #3 Nephrogenic systemic fibrosis

  https://johnsonmemorial.org/jmh-health/disease-conditions/con-20166959

  Diagnosis of nephrogenic systemic fibrosis is made with: […] Physical exam for signs and symptoms of the disease, and evaluation for a possible history of MRI using a gadolinium-based contrast agent when advanced kidney disease is present […] A sample of tissue (biopsy) taken from the skin and muscle […] Other tests as needed that may indicate involvement of muscles and internal organs.

• #3 Nephrogenic Systemic Fibrosis – MD Searchlight

  https://mdsearchlight.com/medications/nephrogenic-systemic-fibrosis/

  Nephrogenic systemic fibrosis (NSF) is a condition seen in patients with kidney problems who have been exposed to a type of contrast agent used in MRI scans called gadolinium. […] Nephrogenic systemic fibrosis (NSF) is a medical condition that cant be confirmed with a specific type of test. […] A common scenario among people with this condition involves significant kidney issues (specifically, when the eGFR kidney function test shows less than 30 mL/min/1.73 m^2) and exposure to a type of contrast agent, known as GBCAs, used in MRI scans. […] A tissue sample from the affected area, such as the skin, with typical disease characteristics can help support the diagnosis of NSF. […] In order to get this tissue sample, a deep skin biopsy is usually performed. […] When examining these samples under a microscope, they could see several changes that suggest NSF.

• #3 Gadolinium Contrast for MRI scans

  https://radiologyacrossborders.org/diagnostic_imaging_pathways/about-imaging/contrast-agents/gadolinium-contrast-for-mri-scans

  Gadolinium (Gd) containing contrast agents have recently been associated with the development of Nephrogenic Systemic Fibrosis (NSF) and should be used with caution in patients with renal failure pending further study. […] Diagnosis is confirmed by deep skin biopsy which demonstrates thickened collagen bundles with surrounding clefts, mucin deposition and proliferation of fibroblasts and elastic fibres. Signs of inflammation are absent histologically. […] The US Food and Drug Administration (FDA) issued a Public Health Advisory warning in June 2006 following a notification by the Danish Health Authority of 25 cases of NSF following gadodiamide exposure. […] Approximately 1600 cases of NSF have been reported to the US Food and Drug Administration (FDA). […] The factor common to all patients developing NSF is renal impairment, especially end-stage kidney disease.

• #3 Nephrogenic Systemic Fibrosis | Musculoskeletal Key

  https://musculoskeletalkey.com/nephrogenic-systemic-fibrosis/

  The majority of patients with NSF develop skin changes within three months of receiving a gadolinium-containing contrast agent. […] The vast majority of cases of NSF have occurred in patients with stage 5 chronic kidney disease (GFR 15 mL/min/1.73 m2 or requiring dialysis). Thus, other conditions should be considered in the differential diagnosis, especially when a patient has lesser degrees of renal dysfunction. […] Although the histological appearance of NSF may be indistinguishable from that of scleromyxedema, the numerous firm, minute papules of scleromyxedema occur on the face and neck, as well as on the hands, arms, and upper trunk. Patients with NSF lack the facial involvement and paraproteinemia typically associated with scleromyxedema. […] Unlike scleroderma, NSF does not involve the face and is not associated with Raynauds phenomenon or circulating autoantibodies.

• #3 Nephrogenic Systemic Fibrosis | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/25703

  Nephrogenic systemic fibrosis (NSF) is a progressive multiorgan fibrosing condition caused by exposure to gadolinium-based contrast agents (GBCAs) used for magnetic resonance imaging (MRI) in the setting of low glomerular filtration rate (GFR). […] NSF occurs in patients with acute or severe chronic renal failure, usually stage 4 or 5 chronic kidney disease (CKD), but it has also been described in patients with stage 3 CKD. […] The condition was first described in 2000 when several physicians across the United States (US) noted patients with a scleroderma-type illness, and the association with GBCA administration in the setting of renal failure was solidified as more cases were presented. […] The risk of developing NSF depends on the amount of residual renal function and the type of GBCA used.

• #3 Nephrogenic Systemic Fibrosis: Background, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/1097889-overview

  The pathophysiology of nephrogenic systemic fibrosis is related to the exposure of patients with renal insufficiency to gadolinium in association with imaging studies. […] The safety of gadopentetate linear product might be no different from macrocyclic preparations such as gadodiamide (Omniscan), but guidelines should be followed in all gadolinium products. […] The cause of nephrogenic systemic fibrosis is the connexation of renal insufficiency and gadolinium exposure from imaging studies. […] Gadolinium-based contrast agents (Ablavar, Eovist, Magnevist, MultiHance, Omniscan, OptiMARK, ProHance) have recently been linked to the development of nephrogenic systemic fibrosis. […] On September 9, 2010, the FDA announced it is requiring that gadolinium-based contrast agents (GBCAs) carry new warnings on their labels about the risk for nephrogenic systemic fibrosis and its association when administered to certain patients with kidney disease.

• #3 Gadolinium Contrast for MRI scans

  https://radiologyacrossborders.org/diagnostic_imaging_pathways/about-imaging/contrast-agents/gadolinium-contrast-for-mri-scans

  Gadolinium exposure has preceded the onset of NSF symptoms by few days to three years with a median interval of 62 days. […] Several reports suggest a dose-response relationship. […] The European Medicines Agency has classified GBCAs into high, intermediate and low risk for NSF. […] For patients with end-stage or chronic or severe or moderate renal impairment or acute kidney injury, Group 1 / high risk GBCAs are contraindicated. […] If GBCAs are essential use a low-risk macrocyclic GBCA, use the lowest possible dose to achieve a diagnostic quality scan. […] Monitor for signs and symptoms of NSF after a GBCA is administered to a patient suspected or known to have impaired elimination of the drug.

• #3 Renal function, nephrogenic systemic fibrosis and other adverse reactions associated with gadolinium-based contrast media | Nefrología

  https://www.revistanefrologia.com/en-renal-function-nephrogenic-systemic-fibrosis-articulo-X2013251414054390

  There is no evidence of effective treatment and only in transplant patients has an improvement or a detention in the progression of renal disease been achieved in the case of acute renal failure. […] The classification of chronic kidney disease (CKD) followed the initial publication of the National Kidney Foundation through the Kidney Disease Outcomes Quality Initiative (K-DOQI) guidelines. […] The definition of CKD by K-DOQI is as follows: Renal damage for at least three months, defined by structural or functional abnormalities of the kidney or without a decrease in the GFR and shown by pathological changes or renal damage markers. […] The reporting of this delayed and potentially severe adverse reaction marked a turning point, since it made it compulsory to establish consensuses to protect patients by assessing the GFR and risk factors.

• #3 15. Nephrogenic Systemic Fibrosis | Hospital Handbook

  https://hospitalhandbook.ucsf.edu/15-nephrogenic-systemic-fibrosis/15-nephrogenic-systemic-fibrosis

  A progressive disease in patients with advanced kidney dysfunction caused by gadolinium contrast used in MRI, leading to dramatic, painful skin thickening due to proliferation of fibrocytes and collagen deposition. Fibrosis of lung, skeletal muscle, liver, testes and myocardium has also been reported. […] NSF is thought to result from gadolinium toxicity after administration and prolonged excretion of gadolinium-based contrast agents (GBCAs) with less-stable gadolinium ions. […] Most cases of have occurred in persons with eGFR 15 or those requiring maintenance dialysis. […] Diagnosis should be confirmed by full thickness skin biopsy and scoring against clinical and histological criteria. […] There is currently no treatment. […] Avoidance of gadolinium exposure in at-risk patients is the only effective strategy. […] Kidney transplantation has been reported to partially reverse the disease.

• #3 Nephrogenic systemic fibrosis – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/nephrogenic-systemic-fibrosis/symptoms-causes/syc-20352299

  Nephrogenic systemic fibrosis can begin days to months, and even years, after exposure to an older gadolinium-based contrast agent (group 1). […] The exact cause of nephrogenic systemic fibrosis isn’t fully understood. […] Exposure to older gadolinium-based contrast agents (group 1) during magnetic resonance imaging (MRI) has been identified as a trigger for development of this disease in people with kidney disease. […] The Food and Drug Administration (FDA) recommends avoiding older gadolinium-based contrast agents (group 1) in people with acute kidney injury or chronic kidney disease. […] Avoidance of older gadolinium-based contrast agents (group 1) is key to preventing nephrogenic systemic fibrosis, as newer gadolinium-based contrast agents (group 2) are safer and are not associated with increased risk.

• #3 SciELO Brazil – Nephrogenic systemic fibrosis: concepts and perspectives Nephrogenic systemic fibrosis: concepts and perspectives

  https://www.scielo.br/j/abd/a/YRrkNwbg8vdYmP5SR85wmcG/?lang=en

  Nephrogenic systemic fibrosis is a chronic, progressive condition that develops in some patients with renal impairment after exposure to gadolinium-based contrast agents used in magnetic resonance imaging. […] The diagnosis of the disease requires careful clinicopathological correlation. […] The diagnosis must be established by a correlation between clinical, laboratory and histopathological findings. The presence of fibrotic skin changes in a patient with renal impairment should prompt further investigation. […] The diagnosis is confirmed by anatomopathological examination and the findings vary according to the stage of evolution of the disease. […] There are no laboratory and pathognomonic histopathological findings or findings consistently associated with the disease that allow its diagnosis in isolation.

• #3 Nephrogenic Systemic Fibrosis: Causes & Symptoms

  https://www.medicoverhospitals.in/diseases/nephrogenic-systemic-fibrosis/

  Diagnosing NSF involves a combination of clinical evaluation, patient history, and laboratory tests. Since NSF shares similarities with other fibrotic and skin conditions, differential diagnosis is essential. […] A thorough clinical examination is the first step in diagnosing NSF, focusing on skin changes and systemic symptoms. Skin biopsy remains a critical diagnostic tool, revealing increased collagen deposition and fibroblast activity indicative of NSF. […] Imaging studies, including MRI and ultrasound, can assess the extent of fibrosis in internal organs. Laboratory tests may include renal function evaluation, inflammatory markers, and analysis of gadolinium levels in the body. […] Diagnosis typically involves clinical evaluation and history of gadolinium exposure.

• #3 Nephrogenic Systemic Fibrosis – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK567754/

  Nephrogenic systemic fibrosis (NSF) is a rare yet serious fibrosing disorder primarily affecting patients with impaired renal function who have been exposed to gadolinium-based contrast agents during magnetic resonance imaging. […] Early recognition of NSF and preventive measures, particularly avoiding certain gadolinium agents in at-risk patients, is critical for reducing its incidence and associated morbidity. […] Participants learn to assess patients’ risk before imaging and recognize NSF symptoms for prompt intervention. […] The course emphasizes an interprofessional approach, promoting collaboration between radiologists, nephrologists, dermatologists, and primary care providers to ensure safe imaging practices and effective, multidisciplinary management for at-risk patients. […] Nephrogenic systemic fibrosis (NSF) is a progressive multiorgan fibrosing condition caused by exposure to gadolinium-based contrast agents (GBCAs) used for magnetic resonance imaging (MRI) in the setting of low glomerular filtration rate (GFR).

• #4 Nephrogenic Systemic Fibrosis in Denmark– A Nationwide Investigation | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0082037

  The patient was diagnosed with NSF if clinical and pathological observations were consistent with the established diagnostic criteria. […] The present nationwide investigation was challenged by several factors. […] The rise in the number of reported NSF cases and subsequent pressure from the media made a nationwide investigation mandatory in Denmark. […] The outcome of the nationwide investigation revealed 65 NSF patients in a population of 5.6 million inhabitants in Denmark, or 12 per million. This high prevalence of NSF worldwide may be rooted in a high level of awareness of NSF both among Danish doctors, politicians and, not least, the media, combined with the fact that Denmark initiated the first nationwide NSF investigation.

• #4 Gadolinium Contrast for MRI scans

  https://radiologyacrossborders.org/diagnostic_imaging_pathways/about-imaging/contrast-agents/gadolinium-contrast-for-mri-scans

  Gadolinium exposure has preceded the onset of NSF symptoms by few days to three years with a median interval of 62 days. […] Several reports suggest a dose-response relationship. […] The European Medicines Agency has classified GBCAs into high, intermediate and low risk for NSF. […] For patients with end-stage or chronic or severe or moderate renal impairment or acute kidney injury, Group 1 / high risk GBCAs are contraindicated. […] If GBCAs are essential use a low-risk macrocyclic GBCA, use the lowest possible dose to achieve a diagnostic quality scan. […] Monitor for signs and symptoms of NSF after a GBCA is administered to a patient suspected or known to have impaired elimination of the drug.

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