Materiały źródłowe

• #1 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: Thickening and hardening of the skin overlying the extremities and trunk; Marked expansion and fibrosis of the dermis in association with CD34+ fibrocytes. […] 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. […] Because of the systemic findings, NSF is preferred to NFD. […] 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 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/print

  Nephrogenic systemic fibrosis (NSF) is a disorder seen only in patients with advanced kidney disease and is characterized by two primary features: Thickening and hardening of the skin overlying the extremities and trunk […] Marked expansion and fibrosis of the dermis in association with CD34+ fibrocytes. 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. Because of the systemic findings, NSF is preferred to NFD. 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.

• #3 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. […] NSF causes your skin to become thick, hard or begin to swell and itch. It can lead to joint contracture, which is when your muscles and tendons become stuck in a bent or straightened position. NSF can also affect other parts of your body like your heart and lungs because it causes the tissue and muscles in your chest to harden. Theres currently no treatment for NSF that can stop its progression or reverse its effects. […] Currently, there are no approved treatments for NSF. There are limited studies on treatments for NSF since the disease is rare. But, the following treatments have been shown to help certain people with NSF: Dialysis can remove gadolinium from your body after an MRI. This can be helpful for people with chronic kidney disease, but theres no evidence that this will stop you from developing NSF. Physical therapy may help preserve your range of motion and flexibility. Kidney transplants could be an option in people with NSF.

• #4 Nephrogenic systemic fibrosis: early recognition and treatment – PubMed

  https://pubmed.ncbi.nlm.nih.gov/18226008/

  Nephrogenic systemic fibrosis (NSF) is a progressive, debilitating, and emotionally distressing disease that can affect patients with renal dysfunction. Prevention, early recognition and early treatment are essential to limiting its impact. The most significant risk factors for developing NSF are chronic or significant acute kidney disease (usually necessitating dialysis) and the administration of gadolinium-containing contrast agents (GCCA). Early symptoms include swelling, redness, pruritus, and pain in the limbs, sometimes with muscle weakness. Early signs are edema, erythema, and occasionally palpable warmth of the involved extremities; there may be florid scleral telangiectasia resembling conjunctivitis. We must redouble our efforts to avoid the administration of GCCA to patients with renal insufficiency. The most effective treatment for NSF to date is maximization of renal function via medical therapy or transplantation. There are data to support a beneficial effect from extracorporeal photopheresis, and all patients can gain from physical therapy.

• #5 Nephrogenic systemic fibrosis (NSF) – Questions and Answers ​in MRI

  https://mriquestions.com/what-is-nsf.html

  Nephrogenic systemic fibrosis (NSF) is a rare, progressive, often fatal disease characterized by skin thickening, painful joint contractures, and fibrosis of multiple organs including the lungs, liver, muscles, and heart. Nearly all documented cases have occurred in patients with chronic severe renal insufficiency who have received gadolinium contrast. The association between gadolinium and NSF was first reported by Danish nephrologists in 2006. Between 2006 and 2010 several hundred cases were diagnosed worldwide. […] NSF usually develops clinically within days to months following gadolinium exposure, although rare cases have been reported years later. Nearly all patients have been in various degrees of renal failure and many were on dialysis. Only exceedingly rare cases have been reported in patients with eGFRs 30 mL/min/1.73m. High and/or multiple doses of contrast are frequently reported, as are the use of linear contrast agents (ACR Group I). Other risk factors include liver disease and acidosis.

• #6 The first case of coexistence of calciphylaxis and nephrogenic systemic fibrosis with different localizations in a hemodialysis patient | Nefrología

  https://revistanefrologia.com/en-the-first-case-coexistence-calciphylaxis-articulo-S2013251420300201

  Nephrogenic systemic fibrosis (NSF) is a rare fibrosing disorder was first described in renally impaired patients as a scleromyxedema-like disorder in 2000 by Cowper et al. It is characterized by progressive diffuse fibrotic changes of the skin bilaterally and symmetrically on the extremities, and occasionally on the trunk. Ninety-five percent of NSF cases are associated with exposure to gadolinium-based contrast agents in renal dialysis patients. […] The patient received hemodialysis treatment with a low calcium dialysate and discontinue a calcium-containing antiphosphate medication. For wound management wet dressing and topical antibiotics were used and morphine-like medications were used for analgesia. […] NSF and calciphylaxis are rare diseases typically seen in chronic renal failure. In literature 95% of NSF cases were associated with exposure to gadolinium-based contrast agents. In our case there was no history of MRI prior to skin lesions, but she had been using erythropoietin (EPO) for some years.

• #7 Nephrogenic systemic fibrosis | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/nephrogenic-systemic-fibrosis

  Nephrogenic systemic fibrosis is a rare disease that occurs mainly in people with advanced kidney failure with or without dialysis. […] Nephrogenic systemic fibrosis can also affect internal organs, such as the heart and lungs, and it can cause a disabling shortening of muscles and tendons in the joints (joint contracture). […] 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. […] The highest risk of nephrogenic systemic fibrosis after exposure to older gadolinium-based contrast agents (group 1) occurs in people who have moderate to severe kidney disease, have had a kidney transplant but have compromised renal function, are receiving hemodialysis or peritoneal dialysis, or have acute kidney injury.

• #8 Nephrogenic Systemic Fibrosis with Multiple Calcification and Osseous Metaplasia | HTML | Acta Dermato-Venereologica

  https://www.medicaljournals.se/acta/content/html/10.2340/00015555-0518

  Nephrogenic systemic fibrosis (NSF) is a rare disease that has been described with increasing frequency in the medical literature. NSF is characterized by skin indurations that affect the extremities in patients with renal failure. […] Nephrogenic systemic fibrosis is now the preferred terminology. […] NSF is characterized clinically by acute onset of hardening and thickening of the skin on the extremities and trunk, often resulting in flexion contractures. It develops only in patients with renal insufficiency. […] Muscle involvement has been reported in severely affected patients. […] One of the typical findings in our case was ossification adjacent to the fascia and muscles in both lower legs. […] In our patient, multiple calcifications in the fascia and muscles were noted along with osseous metaplasia.

• #9 Nephrogenic systemic fibrosis – Humanitas.net

  https://www.humanitas.net/diseases/nephrogenic-systemic-fibrosis/

  Nephrogenic systemic fibrosis (NSF), also called nephrogenis fibrosing dermopathy, is a rare condition of fibrosis of the skin, joints, eyes and internal organs. […] The disease manifests with widely spread patches of thick and hardened skin. […] The disease can also affect the internal organs, such as heart, kidneys and lungs causing shortening of muscle and tendons in the joints or joint contracture. […] When affected by nephrogenic systemic fibrosis people develop large parts of hardened skin with fibrotic nodules and plaques. […] The disease can also lead to joint contracture that causes joint pain and reduces the mobility of the joint. […] Almost 90% of the patients that experienced NSF had an end-stage kidney disease and were on hemodialysis or peritoneal dialysis. […] It seems that an underlying kidney disease is necessary for NSF to appear.

• #10 Nephrogenic Systemic Fibrosis (nephrogenic fibrosing dermopathy, scleromyxedema-like illness of renal disease) – Dermatology Advisor

  https://www.dermatologyadvisor.com/home/decision-support-in-medicine/dermatology/nephrogenic-systemic-fibrosis-nephrogenic-fibrosing-dermopathy-scleromyxedema-like-illness-of-renal-disease/

  Nephrogenic systemic fibrosis is an uncommon condition strongly associated with dosing of gadolinium-containing radiographic contrast materials in the setting of renal disease. […] The disease has been described in all races and in all age groups, and in many countries outside the United States. It seems highly likely that any human with poor renal clearance who is exposed to gadolinium can develop nephrogenic systemic fibrosis. […] It seems that exposure to gadolinium-based material in the setting of acute, chronic, and acute upon chronic renal disease may lead to nephrogenic systemic fibrosis. […] The exact etiology and pathophysiology of NSF is incompletely understood. There is a strong association between the development of NSF and exposure to gadolinium-based radiographic contrast materials during periods of renal insufficiency.

• #11 Nephrogenic systemic fibrosis (NSF) – Questions and Answers ​in MRI

  https://mriquestions.com/what-is-nsf.html

  The strong association with renal insufficiency most likely relates to the prolonged biological half-life due to prolonged excretion of gadolinium. However, other factors have been imputed, including metabolic acidosis; elevated iron and phosphate levels; erythropoietin therapy; vasculopathy; and infectious/inflammatory mediators. […] The pathogenesis of NSF is believed to begin with the displacement of the Gd ion from its chelate by another metallic cation (Fe+3, Zn+2, or Ca+2) through a so-called transmetalation reaction. The free Gd ion is then deposited in the skin and other soft tissues. There it is engulfed by CD163+ iron-recycling and other macrophages creating an inflammatory response and cytokine release. Circulating fibrocytes (immunologically unique CD-34 positive cells derived from bone marrow) deposit in tissue, transforming into spindle cells that proliferate and become the hallmark of the disease. Following recognition of this disorder and its association with gadolinium in patients with renal insufficiency, the worldwide radiology community responded immediately to put an end to this iatrogenic disease. Today, NSF has been nearly completely eliminated due to these measures. In more recent times, however, gadolinium-induced plaques have been reported in the extremities not meeting the full criteria for NSF. The story of NSF is sad one that we radiologists created. It should serve as a lesson that even drugs which appear to be extraordinarily safe may not be infinitely safe for all patients. Sometimes adverse effects may be subtle, disguised, or appear at long time intervals following administration. NSF is thus a call and reminder to be forever vigilant.

• #12 Nephrogenic systemic fibrosis

  https://www.mymlc.com/health-information/diseases-and-conditions/n/nephrogenic-systemic-fibrosis/

  The highest risk of nephrogenic systemic fibrosis after exposure to older gadolinium-based contrast agents (group 1) occurs in people who: Have moderate to severe kidney disease, Have had a kidney transplant but have compromised renal function, Are receiving hemodialysis or peritoneal dialysis, Have acute kidney injury.

• #13 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

  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. […] The first description of what would be known as NSF was published in the year 2000 and consisted of a series of 15 CKD patients from different cities in the United States of America presenting with a scleroderma-like disease. […] Histopathologic examination is essential in the definitive diagnosis of NSF. […] 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.

• #14 :: JKMS :: Journal of Korean Medical Science

  https://jkms.org/DOIx.php?id=10.3346/jkms.2020.35.e293

  Nephrogenic systemic fibrosis (NSF) is a progressive systemic fibrosing disease that may occur after gadolinium contrast exposure. It can lead to severe complications and even death. NSF is highly prevalent among patients with advanced chronic kidney disease (CKD). […] The systemic progression of NSF in the patient stopped when he underwent ultraviolet (UV)-A1 phototherapy and methotrexate (MTX) and steroid therapy. […] The patient underwent intensive physical therapy including stretching, pneumatic compression, and Transcutaneous Electric Nerve Stimulation. […] In conclusion, we described a patient who developed NSF during stage 3a CKD six months after first exposure to gadolinium. When using GBCAs in early stage CKD patients, clinicians should assess the risk factors for AKI and avoid exposure to multiple contrast agents on the same day. In addition, once NSF is suspected following long-term observation, prompt skin biopsy should be performed. In the absence of a definite therapeutic guideline, low dose MTX plus steroid combined with UA-1 phototherapy may be considered as a treatment option for NSF.

• #15 Gadolinium-Associated Nephrogenic Systemic Fibrosis | AAFP

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

  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. […] 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.

• #16

  https://journals.lww.com/nuclearmed/fulltext/2013/03000/nephrogenic_systemic_fibrosis_in_a_patient_with.10.aspx

  Nephrogenic systemic fibrosis (NSF) has been linked to utilization of gadolinium-based contrast agents in patients with renal impairment. […] Incisional biopsy of the left thigh nodule revealed features of NSF with a total pathological score of 4, highly consistent with NSF.

• #17 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. Thickening of the skin is typical, usually affecting the extremities. Visceral organs can also be affected. The diagnosis of the disease requires careful clinicopathological correlation. Treatment aims at restoring renal function, which is associated with delayed progression and, eventually, remission of skin changes. Reduction and prevention of nephrogenic systemic fibrosis cases are based on limiting the use of gadolinium-based contrast agents in patients with kidney disorders (especially in patients with advanced renal failure at stages 4 and 5), and restricting their use to situations in which they are essential to diagnosis/follow-up. Other than limiting exposure to gadolinium based contrast agents, no effective preventive methods have been reported. Due to increased awareness about the disease among radiologists and nephrologists, the incidence of nephrogenic systemic fibrosis is declining.

• #18 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. […] 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. Affected individuals may experience skin discoloration, induration, itchiness, ache, paraesthesia or burning sensation confined to the lower limbs. This will be followed by hardening and thickening of the skin of the extremities and trunk, often resulting in flexion contractures.

• #19 Nephrogenic systemic fibrosis | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/nephrogenic-systemic-fibrosis

  There is no cure for nephrogenic systemic fibrosis, and no treatment is consistently successful in halting or reversing the progression of the disease. […] Certain treatments have shown limited success in some people with nephrogenic systemic fibrosis, but more research is needed to determine if these treatments help: Hemodialysis may decrease the possibility of nephrogenic systemic fibrosis in people with advanced chronic kidney disease who are receiving hemodialysis immediately after receiving a gadolinium-based contrast agent. […] Physical therapy that helps stretch the involved limbs may help slow the progression of joint contractures and preserve movement. […] For people who are appropriate candidates, improvement in renal function because of a kidney transplant may help improve nephrogenic systemic fibrosis over time. […] Some people have shown improvement after receiving extracorporeal photopheresis with ultraviolet A therapy.

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

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

  Our caring team of Mayo Clinic experts can help you with your nephrogenic systemic fibrosis-related health concerns […] Nephrogenic systemic fibrosis care at Mayo Clinic […] Certain treatments have shown limited success in some people with nephrogenic systemic fibrosis, but more research is needed to determine if these treatments help: […] Physical therapy that helps stretch the involved limbs may help slow the progression of joint contractures and preserve movement. […] For people who are appropriate candidates, improvement in renal function because of a kidney transplant may help improve nephrogenic systemic fibrosis over time. […] Some people have shown improvement after receiving this therapy. […] Possible benefit has been shown using this medication, but more research is needed.

• #21 Nephrogenic Systemic Fibrosis Treatment & Management: Medical Care, Surgical Care, Consultations

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

  Nephrogenic systemic fibrosis (NSF) is usually a chronic, progressive condition. Rare cases of partial-to-complete spontaneous resolution have been reported in the absence of specific therapy, with the return of renal function. A favorable response to medical intervention is anecdotal. Of all treatments, extracorporeal photopheresis (ECP) seems to provide the best, albeit mild and extremely expensive, treatment modality for nephrogenic systemic fibrosis. […] Nephrogenic systemic fibrosis does not require inpatient care. No inpatient or outpatient treatment has been particularly successful in treating this condition. Plasmapheresis has shown some promise and is still being evaluated for its efficacy in persons who have not undergone transplantation. […] Surgical care has no role in the treatment of this condition. Although surgical care has no direct role in the treatment of nephrogenic systemic fibrosis, patients who have undergone successful kidney transplantation may show resolution of the lesions. Nephrogenic systemic fibrosis is not a contraindication to transplantation; however, because of reports of associated thrombotic events and early graft loss, evaluation for hypercoagulability should be performed.

• #22 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. […] There is no consistently effective therapy for nephrogenic systemic fibrosis. Adjunct treatment options include improving renal function: renal transplant, recovery from acute renal failure (ARF), physical therapy, extracorporeal photopheresis, plasmapheresis, immunoglobulin therapy, steroids and other immunosuppressive agents.

• #22 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. […] The application of preventive guidelines in the usage of gadolinium-based contrast agents in patients with reduced renal function has been shown to be extremely effective in protecting patients from NSF. […] Patients should be screened for the possible risk of developing nephrogenic systemic fibrosis (NSF) by using institutional screening questionnaires and calculating the eGFR. […] 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.”

• #23 Nephrogenic Systemic Fibrosis with Multiple Calcification and Osseous Metaplasia | HTML | Acta Dermato-Venereologica

  https://www.medicaljournals.se/acta/content/html/10.2340/00015555-0518

  The treatment modalities attempted so far include plasmapheresis, extracorporeal photopheresis, cyclosporin, tacrolimus, intralesional triamcinolone, intralesional methotrexate, interferon-alpha and local PUVA therapy. […] In 2007, Yerram et al. reported a patient with 2 years disease history who demonstrated marked improvement in pain and skin changes after treatment with intravenous STS. […] Correct and expedient diagnosis of NSF may be critical to obtain a better outcome by treating patients in the early stages of the disease.

• #24 Late Onset Nephrogenic Systemic Fibrosis in a Patient with Stage 3 Chronic Kidney Disease: a Case Report

  https://jkms.org/search.php?where=aview&id=10.3346/jkms.2020.35.e293&code=0063JKMS&vmode=PUBREADER

  Nephrogenic systemic fibrosis (NSF) is a progressive systemic fibrosing disease that may occur after gadolinium contrast exposure. It can lead to severe complications and even death. NSF is highly prevalent among patients with advanced chronic kidney disease (CKD). […] The systemic progression of NSF in the patient stopped when he underwent ultraviolet (UV)-A1 phototherapy and methotrexate (MTX) and steroid therapy. […] The restoration of renal function is crucial to prevent the progression of the disease. […] In our case, steroid and MTX in combination with UV-A therapy seemed to relieve the cutaneous symptoms. […] In conclusion, we described a patient who developed NSF during stage 3a CKD six months after first exposure to gadolinium. When using GBCAs in early stage CKD patients, clinicians should assess the risk factors for AKI and avoid exposure to multiple contrast agents on the same day. In addition, once NSF is suspected following long-term observation, prompt skin biopsy should be performed. In the absence of a definite therapeutic guideline, low dose MTX plus steroid combined with UA-1 phototherapy may be considered as a treatment option for NSF.

• #25 Nephrogenic Systemic Fibrosis Treatment & Management: Medical Care, Surgical Care, Consultations

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

  This disease can result in limited movement. Although the role for physical therapy has not been studied, it would appear intuitive to be useful in patients who are affected. […] Because nephrogenic systemic fibrosis may restrict mobility, physical therapy might be helpful for some patients with this condition.

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

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

  Nephrogenic systemic fibrosis (NSF) is the thickening and hardening of your skin, mostly on your arms and legs. Your muscles, joints, organs, and the tissue that covers your brain may also be affected. It is a condition normally found in people with kidney disease. […] The goal of treatment is to improve your kidney function, decrease your pain, and improve your joint movement. You may need any of the following: Prescription pain medicine may be given. Ask your healthcare provider how to safely take this medicine. […] Go to physical therapy. A physical therapist teaches you exercises to help improve movement and strength. Physical therapy can help loosen your tight skin and prevent contractures. A contracture is when your skin or muscles tighten, causing problems with how you move your joints. […] You have the right to help plan your care. Learn about your health condition and how it may be treated. Discuss treatment options with your healthcare providers to decide what care you want to receive. You always have the right to refuse treatment.

• #27 Nephrogenic Systemic Fibrosis (Inpatient Care)

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

  Nephrogenic systemic fibrosis (NSF) is the thickening and hardening of your skin, mostly on your arms and legs. Your muscles, joints, organs, and the tissue that covers your brain may also be affected. It is a condition normally found in people with kidney disease. […] Physical therapy is a program where a therapist teaches you exercises to help improve movement and strength. Physical therapy can help loosen your tight skin and prevent contractures. A contracture is when your skin or muscles tighten, causing problems with how you move your joints. […] You have the right to help plan your care. Learn about your health condition and how it may be treated. Discuss treatment options with your healthcare providers to decide what care you want to receive. You always have the right to refuse treatment.

• #28 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 main objective in the management of patients with NSF is recovery of the renal function, which relates to delay of disease progression, regardless of the treatment adopted. Spontaneous resolution of NSF in renal transplant recipients has been reported. Nevertheless, lesions may remain unchanged after renal transplantation, even if the transplanted organ shows normal function. […] Physiotherapy appears to be useful in preventing and delaying the progression of joint restrictions, as well as in restoring joint mobility. It should be prescribed whenever possible. Better results were obtained with swimming. […] Improvement of renal function is associated with that of skin lesions, which can resolve spontaneously following the restoration of normal renal function. However, the disease is progressive in most cases. So far there are no reports of spontaneous regression of NSF not associated with improvement of renal function. Despite the fact that improvement of fibrotic symptoms is associated with improvement of renal function, there are reports of development of NSF in renal transplant patients after normalization of renal function.

• #29 Nephrogenic Systemic Fibrosis (nephrogenic fibrosing dermopathy, scleromyxedema-like illness of renal disease) – Dermatology Advisor

  https://www.dermatologyadvisor.com/home/decision-support-in-medicine/dermatology/nephrogenic-systemic-fibrosis-nephrogenic-fibrosing-dermopathy-scleromyxedema-like-illness-of-renal-disease/

  While it is not settled definitively, several series have suggested a dose-response relationship in patients with ESRD who are exposed to gadolinium-based contrast and this may be why the first recognized cases occurred after magnetic resonance angiogram, where the dose of contrast used, off-label, was often three-fold more than the dose used in a simple magnetic resonance imaging study. […] Physical therapy, being of some documented benefit, and also being without real negative consequences when professionally managed, would seem an appropriate component of all NSF management strategies. […] Plasmapheresis and extracorporeal photophoresis have been reported effective in several small series, but these modalities are expensive, and could only reasonably be considered for those with strong financial options or by institutions with generous charitable programs.

• #30 Nephrogenic Systemic Fibrosis in a Patient With Multiple Inflammatory Disorders | MDedge

  https://www.mdedge.com/content/nephrogenic-systemic-fibrosis-patient-multiple-inflammatory-disorders

  First described in 2000 in a case series of 15 patients, nephrogenic systemic fibrosis (NSF) is a rare scleroderma-like fibrosing skin condition associated with gadolinium exposure in end stage renal disease (ESRD). Patients with advanced chronic kidney disease (CKD) or ESRD are at the highest risk for this condition when exposed to gadolinium-based contrast dyes. […] Nephrogenic systemic fibrosis is a devastating and rapidly progressive condition, making its prevention in at-risk populations of utmost importance. […] The patient was discharged to continue physical and occupational therapy to preserve his functional mobility, as no other treatment options were available. […] The most effective intervention is early physical therapy to preserve functionality and prevent contracture formation. For patients who are eligible, early renal transplantation may offer the best chance of improved mobility.

• #31 Gadolinium-Induced Nephrogenic Systemic Fibrosis: Classification, Risk and Guidelines

  https://consultqd.clevelandclinic.org/gadolinium-induced-nephrogenic-systemic-fibrosis-classification-risk-and-guidelines

  Nephrogenic systemic fibrosis (NSF) is a debilitating and often-fatal fibrosing disease characterized by skin thickening and organ fibrosis. […] Gadolinium agents that have a linear molecular shape pose a higher risk, and their use was contraindicated in patients with acute and severe chronic kidney disease with eGFRs less than 30 mL/min/1.73 m2, as well as in patients on dialysis. […] In these patients, group I and III gadolinium-based contrast agents are contraindicated, with the caveat that there is insufficient real-life data to determine the risk of NSF from administration of group III agents. […] The guidelines set by the FDA and the radiology societies were undoubtedly effective in curbing the disease and eventually eliminating it. […] The American College of Radiology defines patients at risk of NSF as those: With advanced chronic kidney disease (eGFR 30 mL/min/1.73 m2 not on dialysis), On dialysis (any form), With acute kidney injury.

• #32 Nephrogenic Systemic Fibrosis, Moh’d sharshir | PPT

  https://www.slideshare.net/slideshow/nephrogenic-systemic-fibrosis/101723380

  The major preventive measure for NSF that can be currently recommended to patients with advanced kidney failure is the avoidance of gadolinium. […] The US Food and Drug Administration (FDA) recommends in patients with estimated glomerular filtration rate (eGFR) 30 mL/min/1.73 m2, receiving dialysis, or with acute kidney injury (AKI): Gadolinium-containing contrast agents should be used only if clearly necessary. Gadolinium should be avoided in patients with a diagnosis or clinical suspicion of NSF. […] Among patients with an eGFR 30 mL/min/1.73 m2 who it is felt must receive a gadolinium contrast study, suggest the following approach: The patient should be informed of the benefits, risks, and alternatives. Avoid linear chelates such as gadodiamide, gadoversetamide, and gadopentetate dimeglumine.

• #33 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

  NSF, previously called nephrogenic fibrosing dermopathy (NFD), is a serious and life-threatening condition characterised by the formation of connective tissue in the skin which becomes thickened, coarse, and hard, sometimes leading to contractures and joint immobility. Patients with NSF can have systemic involvement of other organs including the lungs, liver, muscles, and heart. […] 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. It is particularly important to screen patients aged 65 years or older […] renal impairment – for patients with severe renal impairment (glomerular filtration rate [GFR] 30 mL/min/173m2), use of a high-risk agent is contraindicated. If use of a medium-risk agent cannot be avoided or if it is necessary to use a low-risk agent, a single lowest dose possible can be used and should not be repeated for at least 7 days. For patients with moderate renal impairment (GFR 30-59 mL/min/173 m2), if it is necessary to use a high-risk agent a single lowest dose possible can be used and should not be repeated for at least 7 days

• #34 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

  perioperative liver-transplantation period – use of a high-risk agent is contraindicated. If use of a medium-risk agent cannot be avoided or if it is necessary to use a low-risk agent, a single lowest dose possible can be used and should not be repeated for at least 7 days […] neonates – use of a high-risk agent is contraindicated. For medium-risk or low-risk agents, use a single lowest possible dose and do not repeat for at least 7 days […] breastfeeding – discontinue for at least 24 hours after use of a high-risk agent. The decision of whether to continue or suspend breastfeeding for 24 hours after use of a medium-risk or low-risk agent should be at your discretion in consultation with the mother […] pregnancy – use of any gadolinium-containing contrast agent is not recommended unless absolutely necessary.

• #35

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

  No cases of complete remission have been described in the presence of continued renal impairment. […] 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. […] Physiotherapy is reported to maintain mobility in affected joints. […] Extracorporeal photophoresis has among the largest numbers of case series but with mixed results. […] Current guidelines advise against administration of high-risk Gd agents to neonates and advise caution in infants. […] This is based on the hypothesis that immature renal function in neonates and children puts them at increased risk of NSF. […] 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. […] Vigilance when planning MRI, the use of nonenhanced MRI for vascular imaging, and the use of reduced-dose Gd in high-risk children are suggested as important preventative measures.

• #36 Nephrogenic Systemic Fibrosis: Symptoms, Causes, Treatment | Qwark

  https://qwarkhealth.com/conditions/nephrogenic-systemic-fibrosis

  Medications commonly used in the management of NSF include immunosuppressant drugs such as sirolimus, mycophenolate mofetil, and cyclosporine. […] Physical therapy can assist in the treatment of NSF by maintaining mobility and preventing contractures. […] Lifestyle changes that can be implemented to alleviate symptoms of NSF include maintaining a healthy diet, engaging in regular exercise, and avoiding exposure to triggers. […] The prognosis for patients diagnosed with Nephrogenic Systemic Fibrosis (NSF) is variable and dependent on several factors such as the severity of the disease, the extent of organ involvement, and the patient’s overall health condition. […] According to the same study by Marckmann et al. (2008), the fatality rate among patients with moderate NSF involvement was 3.3%, while those with severe disease had a significantly higher mortality rate of about 30%.

• #37

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

  Nephrogenic systemic fibrosis (NSF) is a multisystem disease seen exclusively in patients with renal impairment. It can be severely debilitating and sometimes fatal. […] Although there is no single effective treatment for NSF, the most successful outcomes are seen following restoration of renal function, either following recovery from acute kidney injury or following renal transplantation. […] Continued vigilance is recommended: screening for renal impairment, use of more stable gadolinium chelates, consideration of non-contrast-enhanced MRI or alternative imaging modalities where appropriate. […] Once NSF has developed, the factor most consistently associated with resolution of symptoms is improved renal function. This benefit is seen in patients with chronic renal failure who have undergone successful renal transplantation and in patients whose AKI has resolved.

• #38 Nephrogenic fibrosing dermopathy

  https://dermnetnz.org/topics/nephrogenic-fibrosing-dermopathy

  Many patients die as a complication of their kidney disease or transplant surgery. A minority of patients, approximately 5%, have a fulminant course with rapid progression. Nephrogenic fibrosing dermopathy may contribute to death by restricting mobility leading to falls, fractures and the ensuing complications.

• #39 Nephrogenic Systemic Fibrosis, Moh’d sharshir | PPT

  https://www.slideshare.net/slideshow/nephrogenic-systemic-fibrosis/101723380

  Nephrogenic systemic fibrosis (NSF) is a fibrosing disorder seen only in patients with kidney failure. It is characterized by two primary features: Thickening and hardening of the skin overlying the extremities and trunk. Marked expansion and fibrosis of the dermis in association with CD34- positive fibrocytes. […] NSF occurs exclusively in patients with kidney failure. The first cases were noted between 1997 and 2000 in hemodialysis patients or patients with failed renal allografts who developed severe skin induration that was histologically thought to be scleromyxedema. […] The number of cases has declined dramatically since initial reports, most certainly due to the avoidance of gadolinium-containing agents among at-risk patients. As of January 2013, over 400 cases of NSF had been reported to the International NSF Registry at Yale University. Almost all cases were in adults, but children were also affected.

• #40

  https://journals.lww.com/10.2215/CJN.00140110

  Background and objectives: Nephrogenic systemic fibrosis (NSF) is a disorder that can affect patients with renal dysfunction exposed to a gadolinium-based contrast agent (GBCA). Given the unique role nephrologists play in caring for patients at risk to develop NSF, this study surveyed their perceptions and practices regarding NSF. […] Most nephrologists (56%) felt that enacted policies were appropriate, yet 58% were uncertain if the changes had benefited patients. […] These results indicate that nephrologists are generally familiar with the risk factors and consequences of NSF, but their perceptions do not always align with current evidence. Local policy changes in GBCA use are pervasive. Most nephrologists are comfortable with these policy changes but have mixed feelings regarding their effectiveness.

• #41 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

  Although kidney transplant improves renal function, this may not help to treat NSF. […] 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.

• #42 Nephrogenic Systemic Fibrosis After Gadolinium-Contrast MRIlogo-32logo-40logo-60NEJM Journal WatchnejmJW_1L_RGB-b

  https://www.jwatch.org/na51867/2020/06/30/nephrogenic-systemic-fibrosis-after-gadolinium-contrast

  Nephrogenic systemic fibrosis (NSF) — a severe and often fatal condition caused by collagen deposition in soft tissues and organs — occurs (rarely) in patients after receiving gadolinium-based contrast agents (GBCAs) for magnetic resonance imaging. […] Unfortunately, the number of patients with renal disease who were evaluated after use of newer gadolinium agents was relatively low and likely insufficient to allow definitive assessment of their risk. Although some authorities suggest that NSF cases are nonexistent after newer gadolinium agents are used, this comprehensive analysis suggests that cases might occur occasionally. Prudent use of gadolinium among patients with severely limited renal function is advisable.

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