Materiały źródłowe

• #1 Nephrogenic systemic fibrosis

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

  here is no cure for nephrogenic systemic fibrosis, and no treatment is consistently successful in halting or reversing the progression of the disease. Nephrogenic systemic fibrosis only occurs rarely, making it difficult to conduct large studies. […] 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. In people with advanced chronic kidney disease who are receiving hemodialysis, performing hemodialysis immediately after receiving a gadolinium-based contrast agent may decrease the possibility of nephrogenic systemic fibrosis. […] Physical therapy. Physical therapy that helps stretch the involved limbs may help slow the progression of joint contractures and preserve movement.

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

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

  To date, there are no proven treatments that cure nephrogenic systemic fibrosis, and most often, combination therapy is used. Restoration of renal function, if feasible, is essential to prevent further progression as it appears to slow or hold the progression. In 2005, photodynamic therapy was reported to be a successful treatment option for NSF and has been used since that time with variable success. Ultraviolet (UV) therapy includes UVA, UVB, and UVA with psoralen. […] Extracorporeal photopheresis (ECP) is a promising treatment option for nephrogenic systemic fibrosis. In this procedure, blood is drawn, and the white blood cells are separated and treated with the drug psoralen (8-methoxypsoralen) and UV light. The treated lymphocytes are then infused back into the patient. This is anti-inflammatory without being immunosuppressive. NSF is considered a class 3 indication for ECP „inconclusive evidence.”

• #3

  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. […] 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. […] 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. […] 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.

• #4 Nephrogenic systemic fibrosis – Wikipedia

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

  Multiple therapies for NSF have been attempted, with variable clinical improvement. None have been as effective as restoration of kidney function. Restoration of kidney function by treating the underlying disease process, recovery from acute kidney injury (AKI), or performing a kidney transplant can slow or hold the progression of NSF. A few cases of curative kidney transplantation have been reported, and it is appropriate to consider transplantation as treatment.

• #5 Nephrogenic systemic fibrosis

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

  Kidney transplant. For people who are appropriate candidates, improvement in renal function because of a kidney transplant may help improve nephrogenic systemic fibrosis over time. […] Extracorporeal photopheresis with ultraviolet A. This treatment involves drawing the blood outside the body and treating the blood with a drug that sensitizes it to ultraviolet light. The blood is then exposed to ultraviolet light and returned to the body. Some people have shown improvement after receiving this therapy. […] These medications are experimental, but not currently in use. They have been shown to help some people, but side effects limit their use: […] Imatinib (Gleevec). Although this treatment shows some promise in reducing skin thickening and tightening, more research is needed. […] Pentoxifylline (Pentoxil). There is limited success with this medication, which theoretically decreases the thickness and stickiness (viscosity) of blood, aiding circulation. More research is needed. […] Sodium thiosulfate. Possible benefit has been shown using this medication, but more research is needed. […] High-dose intravenous immune globulin. Possible benefit has been shown using this medication, but more research is needed.

• #6

  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. […] 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. […] 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. […] 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.

• #7 Nephrogenic systemic fibrosis

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

  here is no cure for nephrogenic systemic fibrosis, and no treatment is consistently successful in halting or reversing the progression of the disease. Nephrogenic systemic fibrosis only occurs rarely, making it difficult to conduct large studies. […] 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. In people with advanced chronic kidney disease who are receiving hemodialysis, performing hemodialysis immediately after receiving a gadolinium-based contrast agent may decrease the possibility of nephrogenic systemic fibrosis. […] Physical therapy. Physical therapy that helps stretch the involved limbs may help slow the progression of joint contractures and preserve movement.

• #8 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. […] Treatment aims at restoring renal function, which is associated with delayed progression and, eventually, remission of skin changes. […] 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. […] The therapeutical approaches described are anecdotal and include corticosteroids (topical and systemic), topical vitamin D analogues, other immunomodulators (methotrexate, thalidomide), vascular compression stockings, extracorporeal photopheresis, plasmapheresis and chelates, among others.

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

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

  To date, there are no proven treatments that cure nephrogenic systemic fibrosis, and most often, combination therapy is used. Restoration of renal function, if feasible, is essential to prevent further progression as it appears to slow or hold the progression. In 2005, photodynamic therapy was reported to be a successful treatment option for NSF and has been used since that time with variable success. Ultraviolet (UV) therapy includes UVA, UVB, and UVA with psoralen. […] Extracorporeal photopheresis (ECP) is a promising treatment option for nephrogenic systemic fibrosis. In this procedure, blood is drawn, and the white blood cells are separated and treated with the drug psoralen (8-methoxypsoralen) and UV light. The treated lymphocytes are then infused back into the patient. This is anti-inflammatory without being immunosuppressive. NSF is considered a class 3 indication for ECP „inconclusive evidence.”

• #10 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. […] In 2011, Elmholdt noted that low-dose oral imatinib mesylate slightly improved skin texture but not joint mobility in patients with nephrogenic systemic fibrosis. […] In 2005, Schmook et al reported on successful treatment of nephrogenic systemic fibrosis with photodynamic therapy in a kidney transplant recipient. […] In 2004, Kafi et al found that UV-A1 phototherapy improves nephrogenic systemic fibrosis.

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

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

  To date, there are no proven treatments that cure nephrogenic systemic fibrosis, and most often, combination therapy is used. Restoration of renal function, if feasible, is essential to prevent further progression as it appears to slow or hold the progression. In 2005, photodynamic therapy was reported to be a successful treatment option for NSF and has been used since that time with variable success. Ultraviolet (UV) therapy includes UVA, UVB, and UVA with psoralen. […] Extracorporeal photopheresis (ECP) is a promising treatment option for nephrogenic systemic fibrosis. In this procedure, blood is drawn, and the white blood cells are separated and treated with the drug psoralen (8-methoxypsoralen) and UV light. The treated lymphocytes are then infused back into the patient. This is anti-inflammatory without being immunosuppressive. NSF is considered a class 3 indication for ECP „inconclusive evidence.”

• #12 SSA – POMS: DI 23022.835 – Nephrogenic Systemic Fibrosis – 10/06/2023

  https://secure.ssa.gov/apps10/poms.nsf/lnx/0423022835

  TREATMENT […] 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. […] Steroids and massage therapies may be used to decrease the discomfort associated with skin thickening and connective tissue formation.

• #13 Nephrogenic systemic fibrosis

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

  Kidney transplant. For people who are appropriate candidates, improvement in renal function because of a kidney transplant may help improve nephrogenic systemic fibrosis over time. […] Extracorporeal photopheresis with ultraviolet A. This treatment involves drawing the blood outside the body and treating the blood with a drug that sensitizes it to ultraviolet light. The blood is then exposed to ultraviolet light and returned to the body. Some people have shown improvement after receiving this therapy. […] These medications are experimental, but not currently in use. They have been shown to help some people, but side effects limit their use: […] Imatinib (Gleevec). Although this treatment shows some promise in reducing skin thickening and tightening, more research is needed. […] Pentoxifylline (Pentoxil). There is limited success with this medication, which theoretically decreases the thickness and stickiness (viscosity) of blood, aiding circulation. More research is needed. […] Sodium thiosulfate. Possible benefit has been shown using this medication, but more research is needed. […] High-dose intravenous immune globulin. Possible benefit has been shown using this medication, but more research is needed.

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

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

  To date, there are no proven treatments that cure nephrogenic systemic fibrosis, and most often, combination therapy is used. Restoration of renal function, if feasible, is essential to prevent further progression as it appears to slow or hold the progression. In 2005, photodynamic therapy was reported to be a successful treatment option for NSF and has been used since that time with variable success. Ultraviolet (UV) therapy includes UVA, UVB, and UVA with psoralen. […] Extracorporeal photopheresis (ECP) is a promising treatment option for nephrogenic systemic fibrosis. In this procedure, blood is drawn, and the white blood cells are separated and treated with the drug psoralen (8-methoxypsoralen) and UV light. The treated lymphocytes are then infused back into the patient. This is anti-inflammatory without being immunosuppressive. NSF is considered a class 3 indication for ECP „inconclusive evidence.”

• #15 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. […] In 2011, Elmholdt noted that low-dose oral imatinib mesylate slightly improved skin texture but not joint mobility in patients with nephrogenic systemic fibrosis. […] In 2005, Schmook et al reported on successful treatment of nephrogenic systemic fibrosis with photodynamic therapy in a kidney transplant recipient. […] In 2004, Kafi et al found that UV-A1 phototherapy improves nephrogenic systemic fibrosis.

• #16 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. […] The patient underwent a combined therapy with ultraviolet-A1 phototherapy and methotrexate and steroid therapy for 6 months. The combined therapy stopped the systemic progression of NSF. […] The systemic progression of NSF in the patient stopped when he underwent ultraviolet (UV)-A1 phototherapy and methotrexate (MTX) and steroid therapy. […] Systemic methylprednisolone (20 mg/day) was administered for two weeks to treat the NSF. Additionally, UV-A1 topical phototherapy of 1.5 joule (J)/cm2 was administered to the lower leg lesion twice a week. […] In some case reports, treatments including photopheresis, intravenous immunoglobulin, and local administration of interferon showed limited improvement of skin lesions. […] In our case, steroid and MTX in combination with UV-A therapy seemed to relieve the cutaneous symptoms. […] 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.

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

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

  To date, there are no proven treatments that cure nephrogenic systemic fibrosis, and most often, combination therapy is used. Restoration of renal function, if feasible, is essential to prevent further progression as it appears to slow or hold the progression. In 2005, photodynamic therapy was reported to be a successful treatment option for NSF and has been used since that time with variable success. Ultraviolet (UV) therapy includes UVA, UVB, and UVA with psoralen. […] Extracorporeal photopheresis (ECP) is a promising treatment option for nephrogenic systemic fibrosis. In this procedure, blood is drawn, and the white blood cells are separated and treated with the drug psoralen (8-methoxypsoralen) and UV light. The treated lymphocytes are then infused back into the patient. This is anti-inflammatory without being immunosuppressive. NSF is considered a class 3 indication for ECP „inconclusive evidence.”

• #18 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. […] In 2011, Elmholdt noted that low-dose oral imatinib mesylate slightly improved skin texture but not joint mobility in patients with nephrogenic systemic fibrosis. […] In 2005, Schmook et al reported on successful treatment of nephrogenic systemic fibrosis with photodynamic therapy in a kidney transplant recipient. […] In 2004, Kafi et al found that UV-A1 phototherapy improves nephrogenic systemic fibrosis.

• #19 Nephrogenic systemic fibrosis

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

  Kidney transplant. For people who are appropriate candidates, improvement in renal function because of a kidney transplant may help improve nephrogenic systemic fibrosis over time. […] Extracorporeal photopheresis with ultraviolet A. This treatment involves drawing the blood outside the body and treating the blood with a drug that sensitizes it to ultraviolet light. The blood is then exposed to ultraviolet light and returned to the body. Some people have shown improvement after receiving this therapy. […] These medications are experimental, but not currently in use. They have been shown to help some people, but side effects limit their use: […] Imatinib (Gleevec). Although this treatment shows some promise in reducing skin thickening and tightening, more research is needed. […] Pentoxifylline (Pentoxil). There is limited success with this medication, which theoretically decreases the thickness and stickiness (viscosity) of blood, aiding circulation. More research is needed. […] Sodium thiosulfate. Possible benefit has been shown using this medication, but more research is needed. […] High-dose intravenous immune globulin. Possible benefit has been shown using this medication, but more research is needed.

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

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

  There have been anecdotal reports of improvement in NSF lesions after treatment with the following agents: Methotrexate, Rapamycin, Sodium thiosulfate, Pentoxifylline, Glucocorticoids, Imatinib, Thalidomide, Interferon alpha, Topical calcipotriene, Topical betamethasone, Plasmapheresis, Intravenous immunoglobulin. […] Narcotic analgesics may be required to control pain secondary to contractures. Physical therapy is an important part of the treatment to maintain and improve joint motion and reduce contractures. Renal transplantation may also improve symptoms.

• #21 Nephrogenic systemic fibrosis

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

  Kidney transplant. For people who are appropriate candidates, improvement in renal function because of a kidney transplant may help improve nephrogenic systemic fibrosis over time. […] Extracorporeal photopheresis with ultraviolet A. This treatment involves drawing the blood outside the body and treating the blood with a drug that sensitizes it to ultraviolet light. The blood is then exposed to ultraviolet light and returned to the body. Some people have shown improvement after receiving this therapy. […] These medications are experimental, but not currently in use. They have been shown to help some people, but side effects limit their use: […] Imatinib (Gleevec). Although this treatment shows some promise in reducing skin thickening and tightening, more research is needed. […] Pentoxifylline (Pentoxil). There is limited success with this medication, which theoretically decreases the thickness and stickiness (viscosity) of blood, aiding circulation. More research is needed. […] Sodium thiosulfate. Possible benefit has been shown using this medication, but more research is needed. […] High-dose intravenous immune globulin. Possible benefit has been shown using this medication, but more research is needed.

• #22 Imatinib Appears to Be Beneficial for Nephrogenic Systemic Fibrosislogo-32logo-40logo-60NEJM Journal WatchnejmJW_1L_RGB-b

  https://www.jwatch.org/jd200809120000003/2008/09/12/imatinib-appears-be-beneficial-nephrogenic

  Imatinib appears to have antifibrotic activity. […] Two patients with progressive cutaneous disease and joint contractures due to NSF were treated with imatinib mesylate at a dose of 400 mg/day. […] Both patients improved on both measures within a relatively short period. […] This is the first report of a successful therapy for NSF. […] If these observations are upheld in further studies, imatinib will be a potential therapy for this and other fibrosing disorders.

• #23 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 use of oral prednisone 1mg/kg/day shows conflicting results. […] Patients with recent onset of the disease may show improvement of skin lesions after treatment with thalidomide (50 to 100 mg daily), which apparently does not occur in patients with long-standing disease. […] Reduction of the fibrotic consistency of plaques after extracorporeal photopheresis in patients diagnosed with NSF for less than one year has been described, but patients who had been diagnosed with NSF for a period longer than 12 months showed no response to this therapy. […] High-dose intravenous immunoglobulin showed good results, as well as the use of intravenous sodium thiosulfate in a restricted number of patients, possibly due to their chelating and antioxidant properties. […] Imatinib mesylate (Gleevec – Novartis) inhibits the synthesis of fibronectin and type I collagen by dermal fibroblasts in vitro.

• #24 Nephrogenic systemic fibrosis

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

  Kidney transplant. For people who are appropriate candidates, improvement in renal function because of a kidney transplant may help improve nephrogenic systemic fibrosis over time. […] Extracorporeal photopheresis with ultraviolet A. This treatment involves drawing the blood outside the body and treating the blood with a drug that sensitizes it to ultraviolet light. The blood is then exposed to ultraviolet light and returned to the body. Some people have shown improvement after receiving this therapy. […] These medications are experimental, but not currently in use. They have been shown to help some people, but side effects limit their use: […] Imatinib (Gleevec). Although this treatment shows some promise in reducing skin thickening and tightening, more research is needed. […] Pentoxifylline (Pentoxil). There is limited success with this medication, which theoretically decreases the thickness and stickiness (viscosity) of blood, aiding circulation. More research is needed. […] Sodium thiosulfate. Possible benefit has been shown using this medication, but more research is needed. […] High-dose intravenous immune globulin. Possible benefit has been shown using this medication, but more research is needed.

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

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

  There have been anecdotal reports of improvement in NSF lesions after treatment with the following agents: Methotrexate, Rapamycin, Sodium thiosulfate, Pentoxifylline, Glucocorticoids, Imatinib, Thalidomide, Interferon alpha, Topical calcipotriene, Topical betamethasone, Plasmapheresis, Intravenous immunoglobulin. […] Narcotic analgesics may be required to control pain secondary to contractures. Physical therapy is an important part of the treatment to maintain and improve joint motion and reduce contractures. Renal transplantation may also improve symptoms.

• #26 Nephrogenic systemic fibrosis

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

  Kidney transplant. For people who are appropriate candidates, improvement in renal function because of a kidney transplant may help improve nephrogenic systemic fibrosis over time. […] Extracorporeal photopheresis with ultraviolet A. This treatment involves drawing the blood outside the body and treating the blood with a drug that sensitizes it to ultraviolet light. The blood is then exposed to ultraviolet light and returned to the body. Some people have shown improvement after receiving this therapy. […] These medications are experimental, but not currently in use. They have been shown to help some people, but side effects limit their use: […] Imatinib (Gleevec). Although this treatment shows some promise in reducing skin thickening and tightening, more research is needed. […] Pentoxifylline (Pentoxil). There is limited success with this medication, which theoretically decreases the thickness and stickiness (viscosity) of blood, aiding circulation. More research is needed. […] Sodium thiosulfate. Possible benefit has been shown using this medication, but more research is needed. […] High-dose intravenous immune globulin. Possible benefit has been shown using this medication, but more research is needed.

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

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

  Topical calcipotriene (Dovonex) under occlusion has resulted in subjective improvement in 2 patients. Calcipotriene plus betamethasone dipropionate (Taclonex) seemingly might have a role in topical treatment for nephrogenic systemic fibrosis. […] Richmond et al noted 8 patients with nephrogenic systemic fibrosis, 5 of whom were treated with ECP for a mean number of 34 treatment sessions over a mean of 8.5 months. Mildly improved skin tightening, range of motion, and/or functional capacity were achieved. […] Yerram et al reported on a patient who had nephrogenic systemic fibrosis and had multiple previous exposures to gadolinium (Gd3+) based MRI studies and experienced a substantial decrease in pain and skin changes after a trial of intravenous sodium thiosulfate. […] In 2008, Kreuter et al found limited effects of UV-A1 phototherapy in 3 patients with nephrogenic systemic fibrosis.

• #28 Nephrogenic Systemic Fibrosis – MD Searchlight

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

  Also, some topical treatments have been reported to give subjective improvements in a few cases, including calcipotriene alone and calcipotriene combined with betamethasone dipropionate. However, another treatment known as cyclophosphamide has not shown to be effective. […] Pain from joint contraction can sometimes be managed with narcotic pain relievers. Physical therapy can play a crucial part in maintaining and improving joint movement and reducing the contractions. […] Surgery to transplant a kidney may also bring improvements or reduce symptoms. In fact, any restoration of kidney function has shown symptomatic improvement. […] There are also other treatments that have been viewed as successful for NSF, such as photodynamic therapy (a type of light therapy), UV-A1 (another type of light therapy), and the use of intravenous immunoglobulin. In fact, in some cases observed, UV light treatment alone also seems to be an effective treatment method. […] Another report has shown an individual with NSF responding positively to intravenous sodium thiosulfate, especially if the person has been exposed to a substance known as gadolinium multiple times.

• #29 Nephrogenic systemic fibrosis

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

  Kidney transplant. For people who are appropriate candidates, improvement in renal function because of a kidney transplant may help improve nephrogenic systemic fibrosis over time. […] Extracorporeal photopheresis with ultraviolet A. This treatment involves drawing the blood outside the body and treating the blood with a drug that sensitizes it to ultraviolet light. The blood is then exposed to ultraviolet light and returned to the body. Some people have shown improvement after receiving this therapy. […] These medications are experimental, but not currently in use. They have been shown to help some people, but side effects limit their use: […] Imatinib (Gleevec). Although this treatment shows some promise in reducing skin thickening and tightening, more research is needed. […] Pentoxifylline (Pentoxil). There is limited success with this medication, which theoretically decreases the thickness and stickiness (viscosity) of blood, aiding circulation. More research is needed. […] Sodium thiosulfate. Possible benefit has been shown using this medication, but more research is needed. […] High-dose intravenous immune globulin. Possible benefit has been shown using this medication, but more research is needed.

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

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

  Also in 2004, Chung and Chung found that nephrogenic systemic fibrosis responded to high-dose intravenous immunoglobulin. […] Wahba et al suggest that UV light therapy has a role in the treatment of nephrogenic systemic fibrosis, based on 2 cases with which they were involved. […] A trial of localized psoralen plus UV-A treatment in one patient produced no improvement. Oral prednisone (60 mg PO qd) has been effective in several cases, but it has been discontinued in some patients because of its adverse effects. […] In 1 of 2 patients, intralesional alpha interferon (3 MU 3 times weekly) improved the skin, although in both patients, it had to be discontinued because of its adverse effects. […] A small series of patients with dual liver/kidney transplants showed marked improvement with plasmapheresis.

• #31 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. […] The patient underwent a combined therapy with ultraviolet-A1 phototherapy and methotrexate and steroid therapy for 6 months. The combined therapy stopped the systemic progression of NSF. […] The systemic progression of NSF in the patient stopped when he underwent ultraviolet (UV)-A1 phototherapy and methotrexate (MTX) and steroid therapy. […] Systemic methylprednisolone (20 mg/day) was administered for two weeks to treat the NSF. Additionally, UV-A1 topical phototherapy of 1.5 joule (J)/cm2 was administered to the lower leg lesion twice a week. […] In some case reports, treatments including photopheresis, intravenous immunoglobulin, and local administration of interferon showed limited improvement of skin lesions. […] In our case, steroid and MTX in combination with UV-A therapy seemed to relieve the cutaneous symptoms. […] 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.

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

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

  There have been anecdotal reports of improvement in NSF lesions after treatment with the following agents: Methotrexate, Rapamycin, Sodium thiosulfate, Pentoxifylline, Glucocorticoids, Imatinib, Thalidomide, Interferon alpha, Topical calcipotriene, Topical betamethasone, Plasmapheresis, Intravenous immunoglobulin. […] Narcotic analgesics may be required to control pain secondary to contractures. Physical therapy is an important part of the treatment to maintain and improve joint motion and reduce contractures. Renal transplantation may also improve symptoms.

• #33 Alefacept Therapy for Nephrogenic Systemic Fibrosis: A Case Series – JDDonline – Journal of Drugs in Dermatology

  https://jddonline.com/articles/alefacept-therapy-for-nephrogenic-systemic-fibrosis-a-case-series-S1545961611P0922X

  Nephrogenic systemic fibrosis (NSF) is a recently described systemic fibrosing disorder that develops in the setting of renal insufficiency. […] Several anecdotal reports of successful treatment have been reported, but there is no consistently efficacious therapy. […] We report the improvement or stabilization of cutaneous disease in three patients with NSF using alefacept therapy. […] Recently, the use of rapamycin in a patient with NSF also produced rapid clinical improvement. […] We report improvement or stabilization of disease in three patients with NSF using alefacept, an fusion protein that blocks the T-cell / antigen-presenting cell interaction. […] Treatment with prednisone (60 mg daily) and mycophenolate mofetil (1,500 mg bid) for four weeks was without effect. Alefacept 15mg weekly and physical therapy were added. After four weeks, there was skin softening and increased joint mobility.

• #34 Alefacept Therapy for Nephrogenic Systemic Fibrosis: A Case Series – JDDonline – Journal of Drugs in Dermatology

  https://jddonline.com/articles/alefacept-therapy-for-nephrogenic-systemic-fibrosis-a-case-series-S1545961611P0922X

  Nephrogenic systemic fibrosis (NSF) is a recently described systemic fibrosing disorder that develops in the setting of renal insufficiency. […] Several anecdotal reports of successful treatment have been reported, but there is no consistently efficacious therapy. […] We report the improvement or stabilization of cutaneous disease in three patients with NSF using alefacept therapy. […] Recently, the use of rapamycin in a patient with NSF also produced rapid clinical improvement. […] We report improvement or stabilization of disease in three patients with NSF using alefacept, an fusion protein that blocks the T-cell / antigen-presenting cell interaction. […] Treatment with prednisone (60 mg daily) and mycophenolate mofetil (1,500 mg bid) for four weeks was without effect. Alefacept 15mg weekly and physical therapy were added. After four weeks, there was skin softening and increased joint mobility.

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

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

  There have been anecdotal reports of improvement in NSF lesions after treatment with the following agents: Methotrexate, Rapamycin, Sodium thiosulfate, Pentoxifylline, Glucocorticoids, Imatinib, Thalidomide, Interferon alpha, Topical calcipotriene, Topical betamethasone, Plasmapheresis, Intravenous immunoglobulin. […] Narcotic analgesics may be required to control pain secondary to contractures. Physical therapy is an important part of the treatment to maintain and improve joint motion and reduce contractures. Renal transplantation may also improve symptoms.

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

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

  There have been anecdotal reports of improvement in NSF lesions after treatment with the following agents: Methotrexate, Rapamycin, Sodium thiosulfate, Pentoxifylline, Glucocorticoids, Imatinib, Thalidomide, Interferon alpha, Topical calcipotriene, Topical betamethasone, Plasmapheresis, Intravenous immunoglobulin. […] Narcotic analgesics may be required to control pain secondary to contractures. Physical therapy is an important part of the treatment to maintain and improve joint motion and reduce contractures. Renal transplantation may also improve symptoms.

• #37 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 use of oral prednisone 1mg/kg/day shows conflicting results. […] Patients with recent onset of the disease may show improvement of skin lesions after treatment with thalidomide (50 to 100 mg daily), which apparently does not occur in patients with long-standing disease. […] Reduction of the fibrotic consistency of plaques after extracorporeal photopheresis in patients diagnosed with NSF for less than one year has been described, but patients who had been diagnosed with NSF for a period longer than 12 months showed no response to this therapy. […] High-dose intravenous immunoglobulin showed good results, as well as the use of intravenous sodium thiosulfate in a restricted number of patients, possibly due to their chelating and antioxidant properties. […] Imatinib mesylate (Gleevec – Novartis) inhibits the synthesis of fibronectin and type I collagen by dermal fibroblasts in vitro.

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

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

  There have been anecdotal reports of improvement in NSF lesions after treatment with the following agents: Methotrexate, Rapamycin, Sodium thiosulfate, Pentoxifylline, Glucocorticoids, Imatinib, Thalidomide, Interferon alpha, Topical calcipotriene, Topical betamethasone, Plasmapheresis, Intravenous immunoglobulin. […] Narcotic analgesics may be required to control pain secondary to contractures. Physical therapy is an important part of the treatment to maintain and improve joint motion and reduce contractures. Renal transplantation may also improve symptoms.

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

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

  Topical calcipotriene (Dovonex) under occlusion has resulted in subjective improvement in 2 patients. Calcipotriene plus betamethasone dipropionate (Taclonex) seemingly might have a role in topical treatment for nephrogenic systemic fibrosis. […] Richmond et al noted 8 patients with nephrogenic systemic fibrosis, 5 of whom were treated with ECP for a mean number of 34 treatment sessions over a mean of 8.5 months. Mildly improved skin tightening, range of motion, and/or functional capacity were achieved. […] Yerram et al reported on a patient who had nephrogenic systemic fibrosis and had multiple previous exposures to gadolinium (Gd3+) based MRI studies and experienced a substantial decrease in pain and skin changes after a trial of intravenous sodium thiosulfate. […] In 2008, Kreuter et al found limited effects of UV-A1 phototherapy in 3 patients with nephrogenic systemic fibrosis.

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

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

  There have been anecdotal reports of improvement in NSF lesions after treatment with the following agents: Methotrexate, Rapamycin, Sodium thiosulfate, Pentoxifylline, Glucocorticoids, Imatinib, Thalidomide, Interferon alpha, Topical calcipotriene, Topical betamethasone, Plasmapheresis, Intravenous immunoglobulin. […] Narcotic analgesics may be required to control pain secondary to contractures. Physical therapy is an important part of the treatment to maintain and improve joint motion and reduce contractures. Renal transplantation may also improve symptoms.

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

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

  Topical calcipotriene (Dovonex) under occlusion has resulted in subjective improvement in 2 patients. Calcipotriene plus betamethasone dipropionate (Taclonex) seemingly might have a role in topical treatment for nephrogenic systemic fibrosis. […] Richmond et al noted 8 patients with nephrogenic systemic fibrosis, 5 of whom were treated with ECP for a mean number of 34 treatment sessions over a mean of 8.5 months. Mildly improved skin tightening, range of motion, and/or functional capacity were achieved. […] Yerram et al reported on a patient who had nephrogenic systemic fibrosis and had multiple previous exposures to gadolinium (Gd3+) based MRI studies and experienced a substantial decrease in pain and skin changes after a trial of intravenous sodium thiosulfate. […] In 2008, Kreuter et al found limited effects of UV-A1 phototherapy in 3 patients with nephrogenic systemic fibrosis.

• #42 Nephrogenic Systemic Fibrosis – MD Searchlight

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

  Also, some topical treatments have been reported to give subjective improvements in a few cases, including calcipotriene alone and calcipotriene combined with betamethasone dipropionate. However, another treatment known as cyclophosphamide has not shown to be effective. […] Pain from joint contraction can sometimes be managed with narcotic pain relievers. Physical therapy can play a crucial part in maintaining and improving joint movement and reducing the contractions. […] Surgery to transplant a kidney may also bring improvements or reduce symptoms. In fact, any restoration of kidney function has shown symptomatic improvement. […] There are also other treatments that have been viewed as successful for NSF, such as photodynamic therapy (a type of light therapy), UV-A1 (another type of light therapy), and the use of intravenous immunoglobulin. In fact, in some cases observed, UV light treatment alone also seems to be an effective treatment method. […] Another report has shown an individual with NSF responding positively to intravenous sodium thiosulfate, especially if the person has been exposed to a substance known as gadolinium multiple times.

• #43 Nephrogenic Systemic Fibrosis – DoveMed

  https://www.dovemed.com/diseases-conditions/nephrogenic-systemic-fibrosis?trendmd_shared=1

  Nephrogenic Systemic Fibrosis is generally difficult to treat. […] Newer monoclonal antibody treatments have been found to be helpful in some individuals. […] Symptomatic treatment may be considered based on the organ affected. […] Some treatment measures to improve comfort for the skin signs and symptoms may include: Maintain cleanliness and body hygiene, Using moisturizers or other lotions to prevent the skin from drying, Use of topical steroidal creams and lotions, Antibiotics may be used to treat infections. […] Undertaking treatment of any underlying disorders or conditions.

• #44 Nephrogenic systemic fibrosis

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

  here is no cure for nephrogenic systemic fibrosis, and no treatment is consistently successful in halting or reversing the progression of the disease. Nephrogenic systemic fibrosis only occurs rarely, making it difficult to conduct large studies. […] 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. In people with advanced chronic kidney disease who are receiving hemodialysis, performing hemodialysis immediately after receiving a gadolinium-based contrast agent may decrease the possibility of nephrogenic systemic fibrosis. […] Physical therapy. Physical therapy that helps stretch the involved limbs may help slow the progression of joint contractures and preserve movement.

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

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

  There have been anecdotal reports of improvement in NSF lesions after treatment with the following agents: Methotrexate, Rapamycin, Sodium thiosulfate, Pentoxifylline, Glucocorticoids, Imatinib, Thalidomide, Interferon alpha, Topical calcipotriene, Topical betamethasone, Plasmapheresis, Intravenous immunoglobulin. […] Narcotic analgesics may be required to control pain secondary to contractures. Physical therapy is an important part of the treatment to maintain and improve joint motion and reduce contractures. Renal transplantation may also improve symptoms.

• #46 Nephrogenic systemic fibrosis

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

  here is no cure for nephrogenic systemic fibrosis, and no treatment is consistently successful in halting or reversing the progression of the disease. Nephrogenic systemic fibrosis only occurs rarely, making it difficult to conduct large studies. […] 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. In people with advanced chronic kidney disease who are receiving hemodialysis, performing hemodialysis immediately after receiving a gadolinium-based contrast agent may decrease the possibility of nephrogenic systemic fibrosis. […] Physical therapy. Physical therapy that helps stretch the involved limbs may help slow the progression of joint contractures and preserve movement.

• #47 Treatment of Nephrogenic Systemic Fibrosis – NSF – Nephrogenic Fibrosing Dermopathy – NFD

  https://www.youhavealawyer.com/gadolinium/nsf-treatment/

  Nephrogenic Systemic Fibrosis Treatment Options are Limited. Nephrogenic Systemic Fibrosis, also known as Nephrogenic Fibrosing Dermopathy, (NSF/NFD) is a rare disorder which is progressive and often results in permanent problems or possibly even death. There are currently no known Nephrogenic Systemic Fibrosis treatment options which are consistently successful in curing the disorder. Researchers have seen indications that improving kidney function can slow the progression of the symptoms and in some cases lead to a gradual reversal over time. […] According to information contained on the NFD/NSF Research website, some therapies which may or may not have contributed to observed improvements for those with Nephrogenic Systemic Fibrosis (NFS/NFD) include: Oral Steroids (prednisone) – it is not clear whether prednisone affects the NSF disease or the underlying renal disease, but it has shown improvement for some individuals. Topical Dovonex (under occlusion) – use with vascular compression stockings has been reported to show benefits. Extracorporeal photopheresis (ECP) – after several courses of ECP, some patients in Europe reported softening of the affected areas. Plasmapheresis – some have reported slight improvements while others have shown no improvement. Thalidomide – although long term tolerance of the drug may be an issue, there have been reports of subjective improvements. Ultraviolet therapy – PUVA together with Soriatane and prednisone could be helpful for some patients. Physical Therapy (PT) – exercises, deep massage and swimming may slow the progression of joint problems. Kidney transplant – although renal transplant does not necessarily lead to improvement, when kidney function returns to normal, some have reported partial or complete resolution of NSF.

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

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

  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. […] Dialysis cleans your blood when your kidneys cannot. Extra water, chemicals, and waste products are removed from your blood by a dialyzer or dialysis machine. The dialysis machine does this by passing your blood through a filter, then returning it back to you. Ask for more information about dialysis. […] Photopheresis is when your blood is removed and treated with ultraviolet (UV) light and medicine. Blood is then returned to your body. Ask for more information about photopheresis. […] The following list of medications are related to or used in the treatment of this condition. […] Ofev […] Esbriet […] Actimmune […] pirfenidone […] nintedanib.

• #49 Nephrogenic Systemic Fibrosis (NSF) Best Nephrologist In Delhi | Dr Rajesh Goel | Kidney Care Centre

  https://www.kidneycarecentre.in/nephrogenic-systemic-fibrosis-nsf/

  Used to moisturize the skin and reduce symptoms like dryness and irritation. […] Applied to afflicted regions to lessen thickness of the skin and localized irritation. […] NSAIDs like ibuprofen and others can help control pain and minimize inflammation. […] In cases of extreme pain, acetaminophen or stronger painkillers may be required. […] To avoid contractures and preserve joint flexibility. […] To increase general mobility and muscle strength. Occupational therapy: To enhance quality of life and help with daily activities. […] Indications for fasciotomy include severe joint contractures and restricted range of motion. […] To increase joint mobility and reduce pain, the fascia is surgically released. […] Indications for Skin Grafting: Severe fibrosis and ulceration of the skin with extensive skin involvement. […] To improve function and attractiveness, damaged skin is removed and replaced with healthy skin grafts.

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

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

  There have been anecdotal reports of improvement in NSF lesions after treatment with the following agents: Methotrexate, Rapamycin, Sodium thiosulfate, Pentoxifylline, Glucocorticoids, Imatinib, Thalidomide, Interferon alpha, Topical calcipotriene, Topical betamethasone, Plasmapheresis, Intravenous immunoglobulin. […] Narcotic analgesics may be required to control pain secondary to contractures. Physical therapy is an important part of the treatment to maintain and improve joint motion and reduce contractures. Renal transplantation may also improve symptoms.

• #51 Nephrogenic Systemic Fibrosis – DoveMed

  https://www.dovemed.com/diseases-conditions/nephrogenic-systemic-fibrosis?trendmd_shared=1

  Nephrogenic Systemic Fibrosis is generally difficult to treat. […] Newer monoclonal antibody treatments have been found to be helpful in some individuals. […] Symptomatic treatment may be considered based on the organ affected. […] Some treatment measures to improve comfort for the skin signs and symptoms may include: Maintain cleanliness and body hygiene, Using moisturizers or other lotions to prevent the skin from drying, Use of topical steroidal creams and lotions, Antibiotics may be used to treat infections. […] Undertaking treatment of any underlying disorders or conditions.

• #52 Nephrogenic Systemic Fibrosis | IJNRD

  https://www.dovepress.com/the-clinical-manifestations-and-efficacy-of-different-treatments-used–peer-reviewed-fulltext-article-IJNRD

  The findings in this study provide a quantitative measurement of NSFs presentations and treatment efficacies. This serves to make way for researchers to form comprehensive guidelines on the presentation-based treatment of NSF. […] We have found that the most used treatments for the disease are UV-therapy, renal transplant, and extracorporeal photopheresis, these treatments were used in 13.4% of the patients each. UV-therapy yielded improvement in 81.8% of the patients, renal transplant in 80.0% of the patients, and extracorporeal photopheresis in 81.8% of the patients. Modality of treatment used was significantly related to outcome (p=0.015).

• #53 Nephrogenic Systemic Fibrosis | IJNRD

  https://www.dovepress.com/the-clinical-manifestations-and-efficacy-of-different-treatments-used–peer-reviewed-fulltext-article-IJNRD

  The findings in this study provide a quantitative measurement of NSFs presentations and treatment efficacies. This serves to make way for researchers to form comprehensive guidelines on the presentation-based treatment of NSF. […] We have found that the most used treatments for the disease are UV-therapy, renal transplant, and extracorporeal photopheresis, these treatments were used in 13.4% of the patients each. UV-therapy yielded improvement in 81.8% of the patients, renal transplant in 80.0% of the patients, and extracorporeal photopheresis in 81.8% of the patients. Modality of treatment used was significantly related to outcome (p=0.015).

• #54 Nephrogenic Systemic Fibrosis | IJNRD

  https://www.dovepress.com/the-clinical-manifestations-and-efficacy-of-different-treatments-used–peer-reviewed-fulltext-article-IJNRD

  The findings in this study provide a quantitative measurement of NSFs presentations and treatment efficacies. This serves to make way for researchers to form comprehensive guidelines on the presentation-based treatment of NSF. […] We have found that the most used treatments for the disease are UV-therapy, renal transplant, and extracorporeal photopheresis, these treatments were used in 13.4% of the patients each. UV-therapy yielded improvement in 81.8% of the patients, renal transplant in 80.0% of the patients, and extracorporeal photopheresis in 81.8% of the patients. Modality of treatment used was significantly related to outcome (p=0.015).

• #55 Nephrogenic Systemic Fibrosis | IJNRD

  https://www.dovepress.com/the-clinical-manifestations-and-efficacy-of-different-treatments-used–peer-reviewed-fulltext-article-IJNRD

  The findings in this study provide a quantitative measurement of NSFs presentations and treatment efficacies. This serves to make way for researchers to form comprehensive guidelines on the presentation-based treatment of NSF. […] We have found that the most used treatments for the disease are UV-therapy, renal transplant, and extracorporeal photopheresis, these treatments were used in 13.4% of the patients each. UV-therapy yielded improvement in 81.8% of the patients, renal transplant in 80.0% of the patients, and extracorporeal photopheresis in 81.8% of the patients. Modality of treatment used was significantly related to outcome (p=0.015).

• #56 Nephrogenic Systemic Fibrosis | IJNRD

  https://www.dovepress.com/the-clinical-manifestations-and-efficacy-of-different-treatments-used–peer-reviewed-fulltext-article-IJNRD

  The findings in this study provide a quantitative measurement of NSFs presentations and treatment efficacies. This serves to make way for researchers to form comprehensive guidelines on the presentation-based treatment of NSF. […] We have found that the most used treatments for the disease are UV-therapy, renal transplant, and extracorporeal photopheresis, these treatments were used in 13.4% of the patients each. UV-therapy yielded improvement in 81.8% of the patients, renal transplant in 80.0% of the patients, and extracorporeal photopheresis in 81.8% of the patients. Modality of treatment used was significantly related to outcome (p=0.015).

• #57 Nephrogenic Systemic Fibrosis | IJNRD

  https://www.dovepress.com/the-clinical-manifestations-and-efficacy-of-different-treatments-used–peer-reviewed-fulltext-article-IJNRD

  Nephrogenic systemic fibrosis (NSF) is a rare disorder that occurs in association majorly with chronic kidney disease (CKD). […] Although still a relatively new phenomenon, there are several treatment options utilized in the patients with NSF, depending on the extent of disease and renal impairment. Improvement in renal function mostly leads to simultaneous improvement in the symptoms of NSF, but addressing the dermatological manifestation directly might also aid in achieving positive outcomes. Extracorporeal photopheresis, plasmapheresis, phototherapy, sodium thiosulfate, physical therapy, and kidney transplant are few of the many such treatment options. […] The most used treatments were ultraviolet therapy, renal transplant, and extracorporeal photopheresis; in 13.3% of the patients each.

• #58 Nephrogenic systemic fibrosis

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

  here is no cure for nephrogenic systemic fibrosis, and no treatment is consistently successful in halting or reversing the progression of the disease. Nephrogenic systemic fibrosis only occurs rarely, making it difficult to conduct large studies. […] 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. In people with advanced chronic kidney disease who are receiving hemodialysis, performing hemodialysis immediately after receiving a gadolinium-based contrast agent may decrease the possibility of nephrogenic systemic fibrosis. […] Physical therapy. Physical therapy that helps stretch the involved limbs may help slow the progression of joint contractures and preserve movement.

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

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

  Physiotherapy appears to be useful in preventing and delaying the progression of joint restrictions, as well as in restoring joint mobility. […] Even though recent reports show clinical improvement of NSF after kidney transplantation, there are only few cases described and not all the cases have shown benefits from this approach. […] Reports of improvement with all treatment options are anecdotal; thus, there are no specific recommendations for the therapeutic management of these patients so far.

• #60 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 use of oral prednisone 1mg/kg/day shows conflicting results. […] Patients with recent onset of the disease may show improvement of skin lesions after treatment with thalidomide (50 to 100 mg daily), which apparently does not occur in patients with long-standing disease. […] Reduction of the fibrotic consistency of plaques after extracorporeal photopheresis in patients diagnosed with NSF for less than one year has been described, but patients who had been diagnosed with NSF for a period longer than 12 months showed no response to this therapy. […] High-dose intravenous immunoglobulin showed good results, as well as the use of intravenous sodium thiosulfate in a restricted number of patients, possibly due to their chelating and antioxidant properties. […] Imatinib mesylate (Gleevec – Novartis) inhibits the synthesis of fibronectin and type I collagen by dermal fibroblasts in vitro.

• #61 Gadolinium-Associated Nephrogenic Systemic Fibrosis | AAFP

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

  Prevention of nephrogenic systemic fibrosis is the best practice. […] The FDA recommends that gadolinium-based contrast agents be avoided unless the diagnostic information is essential and not available with noncontrast-enhanced MRI. […] The ESUR guideline on nephrogenic systemic fibrosis specifically discusses the different types of gadolinium contrast media, recommending against using gadodiamide, gadopentetate dimeglumine (Magnevist), and gadoversetamide (Optimark) in patients with stage 4 or 5 chronic kidney disease and those patients with reduced renal function who are awaiting liver transplantation.

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

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

  Nephrogenic systemic fibrosis is a rare disease that occurs mainly in people with advanced kidney failure with or without dialysis. […] 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.

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

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

  Nephrogenic systemic fibrosis is a rare disease that occurs mainly in people with advanced kidney failure with or without dialysis. […] 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.

• #64 Nephrogenic Systemic Fibrosis: Causes & Symptoms

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

  The primary strategy for preventing NSF is minimizing the use of GBCAs in patients with kidney impairment. When MRI with contrast is essential, using the lowest effective dose and selecting agents with a lower NSF risk profile is recommended. Regular monitoring of renal function and gadolinium levels can aid in early detection and intervention.

• #65 Nephrogenic Systemic Fibrosis as a Complication after Gadolinium-Containing Contrast Agents: A Rapid Review

  https://www.mdpi.com/1660-4601/18/6/3000

  In patients with a GFR < 30 mL/min, contrast agents with the lowest risk of developing NSF are recommended. [...] The implementation of the new rules and increased awareness of the risk of complications among radiology staff has resulted in a spectacular decrease in the incidence of NSF in recent years. [...] Nevertheless, vigilance needs to be maintained, and further studies and observations on the incidence of this serious complication should be conducted.

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

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

  The guidelines set by the FDA and the radiology societies were undoubtedly effective in curbing the disease and eventually eliminating it. […] In recent years, evidence has been accumulating as to the safety of group II agents. […] On the basis of accumulating evidence, the recent guidelines of the American College of Radiology, the European Society of Urogenital Radiology, and the Canadian Association of Radiologists all permit the use of group II gadolinium-based contrast agents in patients with advanced kidney disease. […] 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. In patients at risk, if a gadolinium-enhanced MRI study is to be performed, a group II agent should be used.

• #67 Nephrogenic Systemic Fibrosis as a Complication after Gadolinium-Containing Contrast Agents: A Rapid Review

  https://www.mdpi.com/1660-4601/18/6/3000

  Nephrogenic systemic fibrosis (NFS) is a generalized disorder occurring in people with kidney failure. […] The aim of this study was to review the literature in available scientific databases on NFS—complication after gadolinium-containing contrast agents. […] The majority of patients were undergoing dialysis. […] Since 2008, the number of reported cases of NSF has decreased significantly. More recent guidelines and reports indicate that not all contrast agents are associated with the same risk of developing NSF. […] Therefore, it is recommended to limit their use, especially in dialyzed patients and patients with a GFR < 30 mL/min. [...] The risk of developing NSF after gadolinium-containing contrast agents is variable. Recent reports indicate that the use of group II contrast agents carries a low risk of complications in patients with renal disease.

• #68 Nephrogenic Systemic Fibrosis: Causes & Symptoms

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

  Currently, there is no cure for NSF, and treatment primarily focuses on managing symptoms and preventing disease progression. A multidisciplinary approach involving nephrologists, dermatologists, and rheumatologists is often necessary. […] Immunosuppressive Agents: Medications such as corticosteroids and methotrexate may help reduce inflammation and slow the progression of fibrosis. […] Antifibrotic Drugs: Drugs that inhibit collagen synthesis, such as pentoxifylline, have shown some promise in managing NSF symptoms. […] Chelating Agents: Although not always effective, chelating agents like sodium thiosulfate may facilitate the removal of gadolinium from the body. […] Physical Therapy: Regular physical therapy can help maintain joint mobility and muscle strength. […] Extracorporeal Photopheresis: This procedure involves the removal and treatment of blood cells with ultraviolet light, potentially modulating the immune response.

• #69 Nephrogenic Systemic Fibrosis (NSF) Best Nephrologist In Delhi | Dr Rajesh Goel | Kidney Care Centre

  https://www.kidneycarecentre.in/nephrogenic-systemic-fibrosis-nsf/

  Increasing the frequency of dialysis sessions can aid hemodialysis patients in lowering their bodys gadolinium levels. […] Changing dialysis schedules to improve gadolinium clearance. […] Providing sufficient nutrients to promote general health and healing is known as nutritional support. […] Sustaining adequate hydrated to promote healthy kidney function and general wellbeing. […] A multidisciplinary strategy is necessary for the treatment of (NSF), including medication, physical therapy, and maybe surgical procedures to manage symptoms and enhance quality of life.

• #70 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. 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. […] If you have kidney disease, its best to avoid exposure to gadolinium. But there may be situations when gadolinium is necessary to get the best images from an MRI. Healthcare providers should weigh the benefit of the MRI against the potential risk of gadolinium exposure. If your provider orders an MRI with contrast agents and you have kidney problems, ask them if youre at risk for NSF.

• #71 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. 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. […] If you have kidney disease, its best to avoid exposure to gadolinium. But there may be situations when gadolinium is necessary to get the best images from an MRI. Healthcare providers should weigh the benefit of the MRI against the potential risk of gadolinium exposure. If your provider orders an MRI with contrast agents and you have kidney problems, ask them if youre at risk for NSF.

• #72 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 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.

• #73 Nephrogenic systemic fibrosis

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

  Kidney transplant. For people who are appropriate candidates, improvement in renal function because of a kidney transplant may help improve nephrogenic systemic fibrosis over time. […] Extracorporeal photopheresis with ultraviolet A. This treatment involves drawing the blood outside the body and treating the blood with a drug that sensitizes it to ultraviolet light. The blood is then exposed to ultraviolet light and returned to the body. Some people have shown improvement after receiving this therapy. […] These medications are experimental, but not currently in use. They have been shown to help some people, but side effects limit their use: […] Imatinib (Gleevec). Although this treatment shows some promise in reducing skin thickening and tightening, more research is needed. […] Pentoxifylline (Pentoxil). There is limited success with this medication, which theoretically decreases the thickness and stickiness (viscosity) of blood, aiding circulation. More research is needed. […] Sodium thiosulfate. Possible benefit has been shown using this medication, but more research is needed. […] High-dose intravenous immune globulin. Possible benefit has been shown using this medication, but more research is needed.

• #74 Gadolinium-Associated Nephrogenic Systemic Fibrosis | AAFP

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

  Prevention of nephrogenic systemic fibrosis is the best practice. […] The FDA recommends that gadolinium-based contrast agents be avoided unless the diagnostic information is essential and not available with noncontrast-enhanced MRI. […] The ESUR guideline on nephrogenic systemic fibrosis specifically discusses the different types of gadolinium contrast media, recommending against using gadodiamide, gadopentetate dimeglumine (Magnevist), and gadoversetamide (Optimark) in patients with stage 4 or 5 chronic kidney disease and those patients with reduced renal function who are awaiting liver transplantation.

• #75 Nephrogenic Systemic Fibrosis as a Complication after Gadolinium-Containing Contrast Agents: A Rapid Review

  https://www.mdpi.com/1660-4601/18/6/3000

  Nephrogenic systemic fibrosis (NFS) is a generalized disorder occurring in people with kidney failure. […] The aim of this study was to review the literature in available scientific databases on NFS—complication after gadolinium-containing contrast agents. […] The majority of patients were undergoing dialysis. […] Since 2008, the number of reported cases of NSF has decreased significantly. More recent guidelines and reports indicate that not all contrast agents are associated with the same risk of developing NSF. […] Therefore, it is recommended to limit their use, especially in dialyzed patients and patients with a GFR < 30 mL/min. [...] The risk of developing NSF after gadolinium-containing contrast agents is variable. Recent reports indicate that the use of group II contrast agents carries a low risk of complications in patients with renal disease.

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