Materiały źródłowe

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

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

  Nephrogenic systemic fibrosis is a disease of fibrosis of the skin and internal organs reminiscent but distinct from scleroderma or scleromyxedema. It is caused by gadolinium exposure used in imaging in patients who have renal insufficiency. […] Nephrogenic systemic fibrosis almost always occurs in patients with renal insufficiency who have had imaging studies (eg, magnetic resonance angiography) with gadolinium, a contrast agent used in imaging studies. […] Patients with nephrogenic systemic fibrosis may develop large areas of indurated skin with fibrotic nodules and plaques. Flexion contractures with an accompanying limitation of range of motion also can occur. […] Nephrogenic systemic fibrosis is a debilitating and sometimes fatal disease. Characteristics include red or dark patches on the skin; burning, itching, swelling, hardening, and tightening of the skin; yellow spots on the whites of the eyes; joint stiffness with trouble moving or straightening the arms, hands, legs, or feet; pain deep in the hip bones or ribs; and muscle weakness.

• #2 Nephrogenic Systemic Fibrosis – StatPearls – NCBI Bookshelf

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

  Nephrogenic systemic fibrosis (NSF) is a rare yet serious fibrosing disorder primarily affecting patients with impaired renal function who have been exposed to gadolinium-based contrast agents during magnetic resonance imaging. This progressive condition involves fibrosis of the skin and connective tissues, leading to painful skin thickening, joint contractures, and decreased mobility. NSF may extend beyond the skin, involving internal organs such as the liver, lungs, heart, and muscles, and potentially causing severe disability or death. […] The condition is characterized by thickening of the skin and subcutaneous tissue in addition to systemic manifestations. The clinical features may also involve skeletal muscle and any fibrous tissue in the body, including the internal organs such as the liver, heart, and lungs. NSF is a clinically highly variable condition. Some patients may have only mild effects on the skin, but many patients develop significant debility. In some patients, the disease may be lethal.

• #3 Nephrogenic systemic fibrosis | Altru Health System

  https://www.altru.org/health-library/conditions/nephrogenic-systemic-fibrosis

  Nephrogenic systemic fibrosis can begin days to months, and even years, after exposure to an older gadolinium-based contrast agent (group 1). Some signs and symptoms of nephrogenic systemic fibrosis may include: […] The condition is generally long term (chronic), but some people may improve. In a few people, it can cause severe disability, even death. […] In some people, involvement of muscles and body organs may cause: Muscle weakness, Limitation of joint motion caused by muscle tightening (contractures) in arms, hands, legs and feet, Bone pain, particularly in the hip bones or ribs, Reduced internal organ function, including heart, lung, diaphragm, gastrointestinal tract or liver, Yellow plaques on the white surface (sclera) of the eyes.

• #4 Nephrogenic Systemic Fibrosis – StatPearls – NCBI Bookshelf

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

  Clinical manifestations of nephrogenic systemic fibrosis (NSF) may occur days or even years after GBCA exposure but usually present within 2 to 10 weeks. One case report describes a patient on hemodialysis who developed NSF 10 years after his last Gd exposure. […] One of the first and most common manifestations is acute thickening and hardening of the skin. The first noted cases were described as „scleroderma-like.” This condition may develop quickly or slowly. Pruritis and burning sensations are the most common symptoms. Patients may describe tightening or thickening of the skin or that their skin looks shinier. The skin is often lumpy with nodular thickening and indurated plaques with a fluctuating leading edge. […] NSF has also been observed to involve joints and skeletal muscle. Joint involvement usually results in disability and contractures. The palms may have superficial flesh-colored or pink macules or papules, which can coalesce into scaly plaques. Hand and foot blistering has also been noted.

• #5 Gadolinium-Associated Nephrogenic Systemic Fibrosis | AAFP

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

  Nephrogenic systemic fibrosis is a progressive, potentially fatal multiorgan system fibrosing disease related to exposure of patients with renal failure to the gadolinium-based contrast agents used in magnetic resonance imaging. […] Development of nephrogenic systemic fibrosis among patients with severe renal insufficiency following exposure to gadolinium-based contrast agents is approximately 4 percent, and mortality can approach 31 percent. […] Patients develop symptoms of nephrogenic systemic fibrosis from the day of exposure to gadolinium-based contrast agents to several months following. In one study, the median time to symptoms was 11.5 days after exposure. […] The plaques may coalesce to form a peau d’orange appearance. The skin manifestations are symmetric, typically involve the extremities and trunk, and rarely affect the face. Joint contractures are common and often lead to significant disability. The fibrosis is not limited to the skin and can affect multiple organs, leading to multisystem organ failure. Nephrogenic systemic fibrosis also causes respiratory failure from diaphragmatic involvement that may lead to death. Mortality has been reported to be 31 percent, but the true mortality is likely not known because of underreporting and possible misdiagnosis.

• #6 Nephrogenic systemic fibrosis – Wikipedia

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

  Nephrogenic systemic fibrosis is a rare syndrome that involves fibrosis of the skin, joints, eyes, and internal organs. NSF is caused by exposure to gadolinium in gadolinium-based MRI contrast agents (GBCAs) in patients with impaired kidney function. […] Clinical features of NSF develop within days to months and, in some cases, years following exposure to some GBCAs. The main symptoms are the thickening and hardening of the skin associated with brawny hyperpigmentation, typically presenting in a symmetric fashion. The skin gradually becomes fibrotic and adheres to the underlying fascia. The symptoms initiate distally in the limbs and progress proximally, sometimes involving the trunk. Joint contractures of the fingers, elbows and knees can develop secondary to skin involvement and can severely impair physical function.

• #7 Nephrogenic Systemic Fibrosis – MD Searchlight

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

  Symptoms can appear anywhere from a few days to years after exposure, but usually show up within 2-10 weeks. Not all patients have been through dialysis or a kidney transplant, so these treatments arent necessarily a precursor to developing NSF. […] Early signs can include a painful, burning sensation, itching, tiredness, and small raised skin growths or patches often accompanied by swelling. Quite a few patients also have irregularities related to calcium in their bodies. […] The skin may tighten or thicken, leading to stiff hands and possible curling of the fingers. Some people may also observe that their skin looks glossier. These symptoms usually develop slowly. For many, the most noticeable symptoms can cause significant discomfort including pain or itching. […] As NSF advances, the skin might thicken and become darker in color. This can happen on both lower and upper parts of the body, spread from the hands and feet inwards, but it typically doesnt affect the face. The skin can be shiny and hard to touch, sometimes described as having a woody feel. Little bumps and firm lumps under the skin can occur. The skin can also resemble the texture of an orange peel, and patches of thickened skin might have an irregular, creeping border.

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

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

  Nephrogenic systemic fibrosis (NSF) is a rare disease that occurs mostly in people with kidney disease after exposure to gadolinium-based contrast agents (GBCAs). 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. […] Symptoms of NSF include: Swelling, tightening and hardening of your skin. This occurs mostly on your arms, legs and trunk area. It rarely affects your face. Darkened bumps or patches on your skin. Your skin may eventually dimple and resemble an orange peel. Itchy, burning or painful skin. Being unable to move your muscles and joints as you typically can. This is due to your skin thickening and swelling.

• #9 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. […] 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. […] 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.

• #10 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. […] What are the signs and symptoms of NSF? Painful or itchy skin. Reddish bumps on your skin. Thick, hard, or dimpled skin, like an orange peel. Yellow spots or redness on the whites of your eyes. Hair loss. Swelling of your hands and feet, and pain and muscle weakness in your arms or legs. Chest pain. […] The goal of treatment is to improve your kidney function, decrease your pain, and improve your joint movement.

• #11 15. Nephrogenic Systemic Fibrosis | Hospital Handbook

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

  A progressive disease in patients with advanced kidney dysfunction caused by gadolinium contrast used in MRI, leading to dramatic, painful skin thickening due to proliferation of fibrocytes and collagen deposition. Fibrosis of lung, skeletal muscle, liver, testes and myocardium has also been reported. […] Mean time from gadolinium exposure to onset of symptoms is 25 days. […] Early symptoms: skin discoloration, swelling and pain, primarily in the distal lower extremities. […] Later symptoms: distal swelling of the extremities, then thickening and induration due to collagen deposition. In advanced stages, the fibrosis extends to the trunk. At this stage, joint movement is limited by the thickened skin and IV access becomes difficult. The face is invariably spared. Diagnosis should be confirmed by full thickness skin biopsy and scoring against clinical and histological criteria.

• #12 Gadolinium-Associated Nephrogenic Systemic Fibrosis | AAFP

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

  Nephrogenic systemic fibrosis is a progressive, potentially fatal multiorgan system fibrosing disease related to exposure of patients with renal failure to the gadolinium-based contrast agents used in magnetic resonance imaging. […] Development of nephrogenic systemic fibrosis among patients with severe renal insufficiency following exposure to gadolinium-based contrast agents is approximately 4 percent, and mortality can approach 31 percent. […] Patients develop symptoms of nephrogenic systemic fibrosis from the day of exposure to gadolinium-based contrast agents to several months following. In one study, the median time to symptoms was 11.5 days after exposure. […] The plaques may coalesce to form a peau d’orange appearance. The skin manifestations are symmetric, typically involve the extremities and trunk, and rarely affect the face. Joint contractures are common and often lead to significant disability. The fibrosis is not limited to the skin and can affect multiple organs, leading to multisystem organ failure. Nephrogenic systemic fibrosis also causes respiratory failure from diaphragmatic involvement that may lead to death. Mortality has been reported to be 31 percent, but the true mortality is likely not known because of underreporting and possible misdiagnosis.

• #13 Nephrogenic fibrosing dermopathy

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

  Nephrogenic fibrosing dermopathy predominantly affects the skin, but abnormal fibrosis of the internal organs may also occur, which is then often called nephrogenic systemic fibrosis. […] About five per cent will have a rapidly progressive course. Within weeks of disease onset, these patients become dependent on wheelchairs and may suffer from falls. […] Nephrogenic fibrosing dermopathy is usually a chronic and progressive condition. […] Nephrogenic fibrosing dermopathy is rare and relatively newly described. Its natural history is not well understood. Some patients make a gradual recovery or gain improved mobility, but it is progressive in most. […] 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.

• #14 Nephrogenic Systemic Fibrosis – MD Searchlight

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

  Symptoms can appear anywhere from a few days to years after exposure, but usually show up within 2-10 weeks. Not all patients have been through dialysis or a kidney transplant, so these treatments arent necessarily a precursor to developing NSF. […] Early signs can include a painful, burning sensation, itching, tiredness, and small raised skin growths or patches often accompanied by swelling. Quite a few patients also have irregularities related to calcium in their bodies. […] The skin may tighten or thicken, leading to stiff hands and possible curling of the fingers. Some people may also observe that their skin looks glossier. These symptoms usually develop slowly. For many, the most noticeable symptoms can cause significant discomfort including pain or itching. […] As NSF advances, the skin might thicken and become darker in color. This can happen on both lower and upper parts of the body, spread from the hands and feet inwards, but it typically doesnt affect the face. The skin can be shiny and hard to touch, sometimes described as having a woody feel. Little bumps and firm lumps under the skin can occur. The skin can also resemble the texture of an orange peel, and patches of thickened skin might have an irregular, creeping border.

• #15 Nephrogenic Systemic Fibrosis Following Gadolinium Administration | MDedge

  https://mdedge.com/cutis/article/101126/contact-dermatitis/nephrogenic-systemic-fibrosis-following-gadolinium

  Nephrogenic systemic fibrosis (NSF) is an emerging medical entity in patients with renal disease, which results in progressive cutaneous and systemic fibrosis. […] Patients with NSF demonstrate symmetric dermal and subcutaneous fibrosis evidenced by increasing skin induration on clinical examination. Nephrogenic systemic fibrosis most commonly involves the lower extremities, and after extending to the upper extremities and trunk, it sporadically involves the head and neck. […] The clinical manifestation of NSF begins with edema, followed by marked dermal induration, sclerotic plaques, and joint contractures that can lead to considerable disability. […] Clinically, it is characterized by acute onset of cutaneous hardening and thickening of the extremities and the trunk, often resulting in flexion contractures. There may be varying surface changes such as pigmentation, peau d’orange texture, and shiny sclerosis. Patients often experience unpleasant symptoms such as pain, pruritus, stiffness, and paresthesia.

• #16 Nephrogenic systemic fibrosis: A frivolous entity

  https://www.wjgnet.com/2220-6124/full/v10/i3/29.htm

  Nephrogenic systemic fibrosis (NSF) with GBCAs has been a deterrent for the physician and has led to avoidance of these agents in patients with impaired kidney function. NSF is a progressive debilitating multisystem condition described classically in patients with renal insufficiency exposed to gadolinium contrast media. It is characterized by an induration and hardening of the skin. NSF is described to first involve the extremities and can imperceptibly involve internal organs. Symptoms typically include thickening of the skin with pruritus. Symptoms and signs may progress rapidly, with some patients developing contractures and joint immobility. This may be fatal in a few patients. […] The skin lesions are predominantly erythematous papules or nodules which coalesce to form indurated plaques. The lesions are commonly distributed symmetrically and initially involve the lower extremities. They then spread proximally to involve the upper extremities. Furthermore, the trunk is usually involved, and the head is mostly spared except for a scleral plaque. During evolution, lesions are slightly edematous and erythematous associated with itching and a burning sensation. With time they become hard and woody sometimes assuming a cobblestone appearance. The timing of the appearance of skin lesions following gadolinium-based contrast agents (GBCAs) exposure is controversial. It may vary from a few months to several years (1 d to 10 years). The course of skin lesions is usually unremitting and chronic. However, a rapid progressive fulminant course has also been reported in approximately 5% of patients.

• #17 Nephrogenic systemic fibrosis | Altru Health System

  https://www.altru.org/health-library/conditions/nephrogenic-systemic-fibrosis

  Nephrogenic systemic fibrosis can begin days to months, and even years, after exposure to an older gadolinium-based contrast agent (group 1). Some signs and symptoms of nephrogenic systemic fibrosis may include: […] The condition is generally long term (chronic), but some people may improve. In a few people, it can cause severe disability, even death. […] In some people, involvement of muscles and body organs may cause: Muscle weakness, Limitation of joint motion caused by muscle tightening (contractures) in arms, hands, legs and feet, Bone pain, particularly in the hip bones or ribs, Reduced internal organ function, including heart, lung, diaphragm, gastrointestinal tract or liver, Yellow plaques on the white surface (sclera) of the eyes.

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

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

  Nephrogenic systemic fibrosis (NSF) is a rare disease that occurs mostly in people with kidney disease after exposure to gadolinium-based contrast agents (GBCAs). 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. […] Symptoms of NSF include: Swelling, tightening and hardening of your skin. This occurs mostly on your arms, legs and trunk area. It rarely affects your face. Darkened bumps or patches on your skin. Your skin may eventually dimple and resemble an orange peel. Itchy, burning or painful skin. Being unable to move your muscles and joints as you typically can. This is due to your skin thickening and swelling.

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

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

  Nephrogenic systemic fibrosis is a disease of fibrosis of the skin and internal organs reminiscent but distinct from scleroderma or scleromyxedema. It is caused by gadolinium exposure used in imaging in patients who have renal insufficiency. […] Nephrogenic systemic fibrosis almost always occurs in patients with renal insufficiency who have had imaging studies (eg, magnetic resonance angiography) with gadolinium, a contrast agent used in imaging studies. […] Patients with nephrogenic systemic fibrosis may develop large areas of indurated skin with fibrotic nodules and plaques. Flexion contractures with an accompanying limitation of range of motion also can occur. […] Nephrogenic systemic fibrosis is a debilitating and sometimes fatal disease. Characteristics include red or dark patches on the skin; burning, itching, swelling, hardening, and tightening of the skin; yellow spots on the whites of the eyes; joint stiffness with trouble moving or straightening the arms, hands, legs, or feet; pain deep in the hip bones or ribs; and muscle weakness.

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

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

  Nephrogenic Systemic Fibrosis (NSF) is a rare disease involving severe thickening and hardening of the skin (fibrosis) overlying the extremities and trunk. […] Symptoms of NSF include painful, burning itching skin, red/dark areas on the skin, skin thickening, edema, loss of flexibility and severe limitations in movement at the joints of the ankles, knees, feet, arms, wrists, and hands, and raised yellow discoloration on sclera (white outer layer of the eyeball). […] NSF is a debilitating and sometimes fatal disease. People with a fulminant (disease developing or progressing suddenly) form of NSF may become wheelchair dependent within weeks due to development of flexion contractures and loss of mobility. Death may result from complications of kidney disease or transplant surgery. In addition, fractures and falls from wheelchair dependency may be fatal.

• #21 Nephrogenic Systemic Fibrosis – MD Searchlight

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

  A study by Todd et al noticed that the 24-month death rate after examination was 48% in patients with skin changes, and 20% in cases where no skin changes were seen. Most patients become reliant on wheelchairs due to stiffness due to the disease within a few weeks of disease starting. A number of patients have reportedly died from falls and similar complications due to issues with moving around. Alongside this, many patients have reported experiencing chronic nerve pain and extreme itchiness. Lastly, patients may also suffer from flexion contractures, a condition where a joint becomes stuck in a bent position, if the disease affects a joint.

• #22 Nephrogenic systemic fibrosis | Altru Health System

  https://www.altru.org/health-library/conditions/nephrogenic-systemic-fibrosis

  Nephrogenic systemic fibrosis can begin days to months, and even years, after exposure to an older gadolinium-based contrast agent (group 1). Some signs and symptoms of nephrogenic systemic fibrosis may include: […] The condition is generally long term (chronic), but some people may improve. In a few people, it can cause severe disability, even death. […] In some people, involvement of muscles and body organs may cause: Muscle weakness, Limitation of joint motion caused by muscle tightening (contractures) in arms, hands, legs and feet, Bone pain, particularly in the hip bones or ribs, Reduced internal organ function, including heart, lung, diaphragm, gastrointestinal tract or liver, Yellow plaques on the white surface (sclera) of the eyes.

• #23 Nephrogenic Systemic Fibrosis – StatPearls – NCBI Bookshelf

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

  Nephrogenic systemic fibrosis is considered a debilitating, progressive disease. This condition causes visceral and cutaneous fibrosis in patients with severe renal insufficiency exposed to GBCAs. Although skin changes associated with NSF may be improved after the restoration of kidney function, especially after recovery from acute kidney injury, this condition does not usually regress spontaneously and almost always progresses relentlessly. The high mortality rate does not come from the cutaneous lesions but rather from visceral fibrosis, particularly in the cardiac and respiratory systems. […] The most debilitating NSF sequelae are the fibrosis of visceral organs such as the heart, lungs, renal tubules, and skeletal muscles. Although visceral fibrosis is often asymptomatic, it may contribute to the development of pulmonary hypertension, cardiomyopathy, and skeletal muscle weakness.

• #24 Nephrogenic systemic fibrosis – Wikipedia

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

  While skin involvement is on the foreground, the process may involve any organ, e.g., the eye, heart, diaphragm, pleura, pericardium, and kidneys, as well as the lungs and liver. […] 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.

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

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

  Musculoskeletal Joint Stiffness and Pain: Again, some of the depressing signs of arthritis are pain and stiffness of the joints and therefore, movement is restricted. […] Reduced Mobility: Both, and contracture, are associated with joint pain and progression of stiffness reducing mobility and ADL. […] Other cardiac issues include restrictive cardiomyopathy, and such can manifest itself in a situation where fibrosis has developed in the heart. […] Therefore, the patients will experience pulmonary fibrosis followed by diaphragmatic fibrosis and respiratory disorder, restricting lung disease. […] All the symptoms related to swallowing and other GI aspects are traced to esophagitis and other Primary Organs of the Digestive System. […] The overproduction of collagen and fibrosis caused by these cells activation leads to the thickening and the increase of hardness of the skin as well as other tissues characteristic of NSF.

• #26 Nephrogenic systemic fibrosis: A frivolous entity

  https://www.wjgnet.com/2220-6124/full/v10/i3/29.htm

  Several case studies have reported the involvement of subcutaneous structures such as fascia, joints, muscle and periarticular tissue and tendons. Involvement of joints can be severe and compromise range of motion with loss of ambulation and confinement to a wheelchair. Deep fibrosis results in flexion contractures of joints mainly in the hands, wrists, knees and ankles. Data from Mendoza et al showed fibrosis of deep visceral organs such as diaphragm muscles, the myocardium including small coronary arterioles, lungs, kidneys and testes. Another analysis by Schieren et al revealed esophageal dysmotility with consecutive liver fibrosis and esophageal varices. Visceral involvement in the form of cardiomyopathy and pulmonary fibrosis was also reported by Swartz et al.

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

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

  In most patients, nephrogenic systemic fibrosis is a progressive condition. Many patients report stabilization and marginal improvement after years with the condition. […] Nephrogenic systemic fibrosis appears linked to increased morbidity and mortality. […] Within weeks of disease onset, many patients become dependent on a wheelchair because of contractures. Several patients have died because of complications from fractures after falls triggered by their mobility problems.

• #28 Nephrogenic fibrosing dermopathy

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

  Nephrogenic fibrosing dermopathy predominantly affects the skin, but abnormal fibrosis of the internal organs may also occur, which is then often called nephrogenic systemic fibrosis. […] About five per cent will have a rapidly progressive course. Within weeks of disease onset, these patients become dependent on wheelchairs and may suffer from falls. […] Nephrogenic fibrosing dermopathy is usually a chronic and progressive condition. […] Nephrogenic fibrosing dermopathy is rare and relatively newly described. Its natural history is not well understood. Some patients make a gradual recovery or gain improved mobility, but it is progressive in most. […] 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.

• #29 Symptoms Nephrogenic Systemic Fibrosis – Diagnosis NSF – Nephrogenic Fibrosing Dermopathy – NFD

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

  In severe cases, individuals diagnosed with NSF/NFD are unable to walk, unable to extend or move the joints of their arms, hands, legs or feet and complain of muscle weakness. […] NSF/NFD can progress slowly over several weeks, or develop over the course of only a few days. According to data reviewed so far, approximately 5% of those with the condition have a rapidly progressive course. […] In many cases, Nephrogenic Systemic Fibrosis is fatal, even though it is not the actual cause of death. It is not uncommon for tightness and contracture around the joints to lead to falls and fractures, and there have been several reported deaths caused by complications. […] NSF/NFD could also contribute to death by causing restricted ventilation or airways as a result of fibrosis (or scarring) of internal organs. Autopsies have revealed internal muscles with significant fibrosis (or scarring) tissue, in addition to the skin tightening/hardening. Internal organs which are commonly affected include the diaphragm, heart, lungs and kidneys.

• #30 Nephrogenic systemic fibrosis: A frivolous entity

  https://www.wjgnet.com/2220-6124/full/v10/i3/29.htm

  Nephrogenic systemic fibrosis (NSF) with GBCAs has been a deterrent for the physician and has led to avoidance of these agents in patients with impaired kidney function. NSF is a progressive debilitating multisystem condition described classically in patients with renal insufficiency exposed to gadolinium contrast media. It is characterized by an induration and hardening of the skin. NSF is described to first involve the extremities and can imperceptibly involve internal organs. Symptoms typically include thickening of the skin with pruritus. Symptoms and signs may progress rapidly, with some patients developing contractures and joint immobility. This may be fatal in a few patients. […] The skin lesions are predominantly erythematous papules or nodules which coalesce to form indurated plaques. The lesions are commonly distributed symmetrically and initially involve the lower extremities. They then spread proximally to involve the upper extremities. Furthermore, the trunk is usually involved, and the head is mostly spared except for a scleral plaque. During evolution, lesions are slightly edematous and erythematous associated with itching and a burning sensation. With time they become hard and woody sometimes assuming a cobblestone appearance. The timing of the appearance of skin lesions following gadolinium-based contrast agents (GBCAs) exposure is controversial. It may vary from a few months to several years (1 d to 10 years). The course of skin lesions is usually unremitting and chronic. However, a rapid progressive fulminant course has also been reported in approximately 5% of patients.

• #31 15. Nephrogenic Systemic Fibrosis | Hospital Handbook

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

  A progressive disease in patients with advanced kidney dysfunction caused by gadolinium contrast used in MRI, leading to dramatic, painful skin thickening due to proliferation of fibrocytes and collagen deposition. Fibrosis of lung, skeletal muscle, liver, testes and myocardium has also been reported. […] Mean time from gadolinium exposure to onset of symptoms is 25 days. […] Early symptoms: skin discoloration, swelling and pain, primarily in the distal lower extremities. […] Later symptoms: distal swelling of the extremities, then thickening and induration due to collagen deposition. In advanced stages, the fibrosis extends to the trunk. At this stage, joint movement is limited by the thickened skin and IV access becomes difficult. The face is invariably spared. Diagnosis should be confirmed by full thickness skin biopsy and scoring against clinical and histological criteria.

• #32 Nephrogenic Systemic Fibrosis – StatPearls – NCBI Bookshelf

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

  Nephrogenic systemic fibrosis is considered a debilitating, progressive disease. This condition causes visceral and cutaneous fibrosis in patients with severe renal insufficiency exposed to GBCAs. Although skin changes associated with NSF may be improved after the restoration of kidney function, especially after recovery from acute kidney injury, this condition does not usually regress spontaneously and almost always progresses relentlessly. The high mortality rate does not come from the cutaneous lesions but rather from visceral fibrosis, particularly in the cardiac and respiratory systems. […] The most debilitating NSF sequelae are the fibrosis of visceral organs such as the heart, lungs, renal tubules, and skeletal muscles. Although visceral fibrosis is often asymptomatic, it may contribute to the development of pulmonary hypertension, cardiomyopathy, and skeletal muscle weakness.

• #33 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. […] NSF usually manifests within 2–10 weeks after the initial exposure; however, clinical manifestations may only become apparent a couple of years after GBCA exposure. […] A high degree of suspicion should be maintained to detect and link these symptoms with a former GBCA exposure. […] NSF severity is graded from 0–4 as follows: 0, asymptomatic; 1, mild physical, dermatologic, or neuropathic symptoms without any kind of disability; 2, moderate physical or neuropathic symptoms limiting physical performance; 3, severe symptoms limiting daily physical activities; and 4, severely disabling symptoms causing dependence on daily activities.

• #34

  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. […] The natural history of NSF is variable. As the condition progresses, the skin may take on a woody texture. Joint contractures or reduced range of movement are seen in 60 % of patients. Of 345 patients in the NSF Registry in 2009, almost 10 % were known to be restricted to a wheelchair. It is estimated that 5 % of patients have a rapidly progressive course, and NSF may increase mortality rates through restricted mobility and, more rarely, restricted ventilation. […] The clinical picture in NSF is very similar to scleromyxedema, but NSF favors the extremities and trunk rather than the head and neck. […] 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. No cases of complete remission have been described in the presence of continued renal impairment.

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

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

  The natural outcome of NSF is not fully known. It has been reported that in up to 5% of cases, it may have a fulminant course. A third will have a mild course without functional limitation. […] There is no evidence of effective treatment and only in transplant patients has an improvement or a detention in the progression of renal disease been achieved in the case of acute renal failure.

• #36 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. […] NSF usually manifests within 2–10 weeks after the initial exposure; however, clinical manifestations may only become apparent a couple of years after GBCA exposure. […] A high degree of suspicion should be maintained to detect and link these symptoms with a former GBCA exposure. […] NSF severity is graded from 0–4 as follows: 0, asymptomatic; 1, mild physical, dermatologic, or neuropathic symptoms without any kind of disability; 2, moderate physical or neuropathic symptoms limiting physical performance; 3, severe symptoms limiting daily physical activities; and 4, severely disabling symptoms causing dependence on daily activities.

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

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

  Fibrosis can progress and cause hardened deposits in your muscles, lungs, esophagus, eyes and heart. This can impact how well these organs work. It can cause long-term and permanent disability and be fatal in some people. […] Complications of NSF can be severe and fatal. Noticing your skin is tight or hard is usually the first sign. But, as the condition worsens, the hardening can affect your internal organs and muscles. For example, your muscles and joints could become so stiff and unmovable that youre unable to walk. If NSF affects your lungs or heart, it can cause breathing problems or impair your hearts ability to pump blood.

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

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

  In most patients, nephrogenic systemic fibrosis is a progressive condition. Many patients report stabilization and marginal improvement after years with the condition. […] Nephrogenic systemic fibrosis appears linked to increased morbidity and mortality. […] Within weeks of disease onset, many patients become dependent on a wheelchair because of contractures. Several patients have died because of complications from fractures after falls triggered by their mobility problems.

• #39 Gadolinium-Associated Nephrogenic Systemic Fibrosis

  https://mobile.fpnotebook.com/Renal/Rad/GdlnmAsctdNphrgncSystmcFbrs.htm

  Onset within 2-3 months of gadolinium exposure (median 11 days) […] Pruritic rash involving symmetric extremities and trunk […] Erythematous Plaques with induration and swelling […] Peau d’orange appearance […] Lesions are tender and may be painful […] Symmetrically distributed lesions on extremities and trunk […] Lower extremities are most involved (but may also affect the arms and torso) […] Spares the face […] Chronic unremitting course […] Mortality: 31%

• #40 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 is a rare disease discovered in 1997, defined as a multiorgan failure that is progressive in nature. It is mainly reported as an iatrogenic disease of the skin as most of the thickening of the skin and subcutaneous tissue arises secondary to exposure to gadolinium-based contrast agents (GBCAs). A strong link between GBCAs and NSF was reported in the early 2000s, with around 500 cases reported from 1997 to 2007. In our study, 79.5% of the patients were exposed to GBCAs. Literature reports that patients with renal insufficiency, especially stage 4 or 5 CKD, are more prone to developing NSF. Similarly, in our study, 88% of the patients had renal impairment. […] The prognosis of this disease and effectivity of the treatment almost depends on the extent of the disease. If the condition progresses from the skin to visceral fibrosis, chances of symptoms regression on the improvement of kidney function decrease. A higher mortality rate is reported in patients with visceral involvement, particularly in the cardiac and respiratory systems. Mortality due to NSF can be due to worsening renal function due to fibrosis of the tubules, cardiomyopathy, or even falls. In our study, the mortality rate was 10.8%. However, only one patient died due to NFS.

• #41 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. This new disease entity can lead to significant disability or even death. The course of the disease is mostly progressive. It may be accompanied by pain, muscle weakness, joint spasms that lead to cachexia, severe disability and, consequently, death. The time from exposure to NSF symptoms was from two days to three years. Most NSF occurs after exposure to linear contrast agents. Therefore, it is recommended to limit their use, especially in dialyzed patients and patients with a GFR < 30 mL/min. [...] The mortality rate within 24 months was significantly higher in patients with NSF and was 48%, while in those with no NSF it was 20%. [...] The common feature of all patients with NSF is renal failure. Many authors emphasized that the highest risk of NSF exists in patients with a GFR < 15 mL/min/1.73 m² (i.e., chronic kidney disease at stage 5). [...] NFS is undoubtedly a serious complication that can lead to significant disability, invalidity and even death.

• #42 Nephrogenic Systemic Fibrosis – StatPearls – NCBI Bookshelf

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

  Nephrogenic systemic fibrosis (NSF) is a rare yet serious fibrosing disorder primarily affecting patients with impaired renal function who have been exposed to gadolinium-based contrast agents during magnetic resonance imaging. This progressive condition involves fibrosis of the skin and connective tissues, leading to painful skin thickening, joint contractures, and decreased mobility. NSF may extend beyond the skin, involving internal organs such as the liver, lungs, heart, and muscles, and potentially causing severe disability or death. […] The condition is characterized by thickening of the skin and subcutaneous tissue in addition to systemic manifestations. The clinical features may also involve skeletal muscle and any fibrous tissue in the body, including the internal organs such as the liver, heart, and lungs. NSF is a clinically highly variable condition. Some patients may have only mild effects on the skin, but many patients develop significant debility. In some patients, the disease may be lethal.

• #43 Nephrogenic Systemic Fibrosis – MD Searchlight

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

  Nephrogenic systemic fibrosis (NSF) is a condition that tends to worsen over time and involves hardening of tissues in multiple organs throughout the body. This usually happens because the patient has been exposed to a special type of contrast dye called gadolinium-based contrast agents (GBCAs). GBCAs are often used during magnetic resonance imaging (MRI) scans. The main symptom of NSF is thickening of the skin and the layer just below the skin, but other symptoms can occur as well, depending on which parts of the body are affected, such as the liver, heart, muscles, and lungs. […] The severity of NSF can vary widely from patient to patient. Some may only see minor changes in their skin, while others could experience limited movement. Its also possible, though rare, that the disease becomes severe enough to be fatal.

• #44 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 typical clinical symptom is hardening of the skin, generally of the extremities but sometimes extending to the trunk. There is associated brawny hyperpigmentation, frequently accompanied by sensory symptoms. Joint contractures are common, and frequently lead to impaired mobility. […] Cutaneous changes develop days to weeks after exposure to GBCA. They are characterized by thickening and hardening of the skin associated with brawny hyperpigmentation, typically presenting in a symmetric fashion. These changes initially affect the distal portion of the lower limbs, progressing proximally, sometimes reaching the trunk. Involvement of the lower limbs typically begins with the talocalcaneal joints, ascending to the central region of the thighs. As for involvement of the upper limbs, the changes initially occur on the wrists, also with ascendant progression to the arms. The face is typically not affected.

• #45 Nephrogenic systemic fibrosis – Wikipedia

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

  Nephrogenic systemic fibrosis is a rare syndrome that involves fibrosis of the skin, joints, eyes, and internal organs. NSF is caused by exposure to gadolinium in gadolinium-based MRI contrast agents (GBCAs) in patients with impaired kidney function. […] Clinical features of NSF develop within days to months and, in some cases, years following exposure to some GBCAs. The main symptoms are the thickening and hardening of the skin associated with brawny hyperpigmentation, typically presenting in a symmetric fashion. The skin gradually becomes fibrotic and adheres to the underlying fascia. The symptoms initiate distally in the limbs and progress proximally, sometimes involving the trunk. Joint contractures of the fingers, elbows and knees can develop secondary to skin involvement and can severely impair physical function.

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

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

  We describe here a 50-year-old Japanese man with nephrogenic systemic fibrosis. He had been suffering from chronic renal insufficiency and had been treated with haemodialysis. He had undergone magnetic resonance angiography using gadodiamide 7 years previously. One month after magnetic resonance angiography, he noted swelling, hotness, induration and pain in his left arm. The same symptoms gradually spread over his lower extremities, resulting in flexion contractures with limited range of motion. Physical examination revealed skin sclerosis on his extremities with a glossy brownish skin surface. […] 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. Histologically, an increase in spindle-shaped cells, collagen fibres, and sometimes mucin deposition in the dermis, have been demonstrated.

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

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

  One of the typical findings in our case was ossification adjacent to the fascia and muscles in both lower legs. Patients with NSF have frequently been reported to show a spectrum of abnormal calcification in the dermis and subcutis, ranging from the expected dystrophic calcinosis cutis, benign nodular calcifications, and metastatic calcinosis cutis that occur in patients with renal disease, to a very unusual case of osseous metaplasia in NSF lesions. […] In our patient, multiple calcifications in the fascia and muscles were noted along with osseous metaplasia. Metaplastic ossification can be regarded as a peculiar histological feature of a subset of NSF patients, especially in the late stages of the disease.

• #48

  https://journals.lww.com/amjdermatopathology/fulltext/2010/02000/nephrogenic_systemic_fibrosis__report_of_an.15.aspx?generateEpub=Article%7Camjdermatopathology:2010:02000:00015%7C10.1097/dad.0b013e3181b0b859%7C

  Nephrogenic systemic fibrosis (NSF) is a novel disease entity described over the past 10 years. NSF is a progressive systemic fibrosing disorder that occurs arguably exclusively in patients with impaired renal function who have been exposed to gadolinium-containing contrast agents. As no single clinical or histopathologic finding is diagnostic of NSF, a careful review of the cumulative characteristics of each case is essential in making a correct diagnosis. The spectrum of histologic variants of NSF continues to expand, including a report of NSF mimicking erythema nodosum and several case reports of NSF with giant cells and calcification. We report an additional case of NSF with the uncommon pathologic features of granulomatous and lymphocytic inflammation in the fibrous septae similar to erythema nodosum.

• #49 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

  After exposure to GBCAs, the time of onset of NSF ranges from a few days to 6 months. In almost all of the cases, NSF is chronic and unremitting in nature. Joint contractures can be so debilitating that patients become wheelchair-bound within days to weeks after onset of the disease. […] Our experience with this patient shows that the early skin features of NSF can be histopathologically indistinguishable from EN. Besides, the acute inflammatory like reaction experienced by the patients post gadodiamide has further aroused the suspicion of EN. Nevertheless, we believe that the patients presentation is within the spectrum of manifestation of NSF.

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

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

  NSF is a multisystemic fibrotic disease that affects the skin, muscle, and other organs (including lung, esophagus, and kidney) described in patients with severe renal impairment exposed to a GBCA. […] The pathophysiology and molecular mechanism of NSF are still a matter of debate. It is believed that the intravenous administration of some GBCAs causes a limited chelate instability that plays an essential role in the release of free Gd. […] After de-chelation (by transmetalation), free Gd binds with endogenous anions, creating an insoluble precipitate that penetrates the interstitial tissue of the lung, esophagus, liver, and kidneys. […] Most GBCAs have preferential renal elimination, and their clearance is highly dependent on the glomerular filtration rate. A small percentage of the administered dose is eliminated via the hepatobiliary route. In CKD patients, the prolonged GBCAs’ half-life leads to a significant release of Gd, and therefore, a higher burden of Gd precipitates.

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

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

  Renal Impairment: Those patients with non-specific fevers, most of them have severe renal failure. […] Oliguria or low output and anuria or no output at all may operational perhaps be due to impaired functionality of the kidneys. […] Consequently, due to often high arterial pressure, chronic renal disease is further complicated by the development of NSF. […] In a state of hyperkalemia, the changes concerning the resting membrane potential put considerable strain on the heart as they jeopardize the normal generation of a population of action potential by cardiac pacemaker cells thus worsening and lead to cardiac arrhythmias. […] Once NSF has affected the skin, they could get dark hence have a hyperpigmentation According to NSF they may change color and become dark. […] It becomes pale and delicate; shrinking can be primary from fingers and toes and secondary from the arms and thighs and the trunk.

• #52 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 is a rare disease discovered in 1997, defined as a multiorgan failure that is progressive in nature. It is mainly reported as an iatrogenic disease of the skin as most of the thickening of the skin and subcutaneous tissue arises secondary to exposure to gadolinium-based contrast agents (GBCAs). A strong link between GBCAs and NSF was reported in the early 2000s, with around 500 cases reported from 1997 to 2007. In our study, 79.5% of the patients were exposed to GBCAs. Literature reports that patients with renal insufficiency, especially stage 4 or 5 CKD, are more prone to developing NSF. Similarly, in our study, 88% of the patients had renal impairment. […] The prognosis of this disease and effectivity of the treatment almost depends on the extent of the disease. If the condition progresses from the skin to visceral fibrosis, chances of symptoms regression on the improvement of kidney function decrease. A higher mortality rate is reported in patients with visceral involvement, particularly in the cardiac and respiratory systems. Mortality due to NSF can be due to worsening renal function due to fibrosis of the tubules, cardiomyopathy, or even falls. In our study, the mortality rate was 10.8%. However, only one patient died due to NFS.

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

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

  There might be joint motion restriction secondary to skin involvement. Joint contractures and motion restrictions may develop within days or weeks, leading to progressive limitation of daily activities and reduced patient’s quality of life. […] Extracutaneous manifestations may occur, and no predictive factors have been identified so far. Telangiectasia and scleral plaques have been reported, but without visual acuity changes. Visceral and skeletal muscles may be affected. Gd deposition in the cardiac tissue can be as high as in the affected skin, which can lead to myocardial fibrosis. […] The prognosis varies depending on the severity of the systemic involvement, extent of skin lesions and time of onset.

• #54

  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. […] The natural history of NSF is variable. As the condition progresses, the skin may take on a woody texture. Joint contractures or reduced range of movement are seen in 60 % of patients. Of 345 patients in the NSF Registry in 2009, almost 10 % were known to be restricted to a wheelchair. It is estimated that 5 % of patients have a rapidly progressive course, and NSF may increase mortality rates through restricted mobility and, more rarely, restricted ventilation. […] The clinical picture in NSF is very similar to scleromyxedema, but NSF favors the extremities and trunk rather than the head and neck. […] 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. No cases of complete remission have been described in the presence of continued renal impairment.

• #55 Nephrogenic Systemic Fibrosis

  https://www.medscape.com/viewarticle/714922

  Extracutaneous manifestations of NSF also occur. Yellow plaques are present on the sclerae of the eyes of many patients with NSF, nasal and temporal to the iris, but do not typically compromise vision. […] Although skin changes have been reported to improve in several patients with NSF upon normalization of kidney function, following renal transplantation or the resolution of acute renal failure, NSF almost always progresses relentlessly and does not usually regress spontaneously. […] NSF is associated with increased mortality. Among patients with stage 5 CKD, those with cutaneous changes of NSF have a threefold increased risk of dying within 2 years of the diagnosis of NSF.

• #56 Nephrogenic systemic fibrosis: the end of the story? – Malikova – Quantitative Imaging in Medicine and Surgery

  https://qims.amegroups.org/article/view/27779/html

  Unfortunately, clinical outcomes or follow-ups were reported only in 341 patients, 110 of them died; however, only in 4 of them their death was attributed directly to NSF. Partial resolution of NSF symptoms was found in 84 patients and even cure of NSF was reported in 12 patients; 40% of them all had acute kidney failure. Improvement of the disease was also reported in females during pregnancy (in 22 subjects) and in some patients after the cessation of -blockers (in 5 subjects). Approximately 18% of cases were reported as a stable disease. […] The second interesting finding is that NSF looks like an age dependent disease, because it was not reported in newborns and toddlers; the youngest reported child was 6 years old. This fact is remarkable, especially when newborns with immature kidneys were often examined with high doses of GBCA. Also, affection of the patients above 80 years were exceptional, only 7 cases were reported by Attari et al. […] Finally, and the most important, only seven NSF cases after GBCA exposures were reported since 2008. This result confirms the huge success of regulations, warnings and recommendations of authorities and radiology societies.

• #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 is a rare disease discovered in 1997, defined as a multiorgan failure that is progressive in nature. It is mainly reported as an iatrogenic disease of the skin as most of the thickening of the skin and subcutaneous tissue arises secondary to exposure to gadolinium-based contrast agents (GBCAs). A strong link between GBCAs and NSF was reported in the early 2000s, with around 500 cases reported from 1997 to 2007. In our study, 79.5% of the patients were exposed to GBCAs. Literature reports that patients with renal insufficiency, especially stage 4 or 5 CKD, are more prone to developing NSF. Similarly, in our study, 88% of the patients had renal impairment. […] The prognosis of this disease and effectivity of the treatment almost depends on the extent of the disease. If the condition progresses from the skin to visceral fibrosis, chances of symptoms regression on the improvement of kidney function decrease. A higher mortality rate is reported in patients with visceral involvement, particularly in the cardiac and respiratory systems. Mortality due to NSF can be due to worsening renal function due to fibrosis of the tubules, cardiomyopathy, or even falls. In our study, the mortality rate was 10.8%. However, only one patient died due to NFS.

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

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

  The natural outcome of NSF is not fully known. It has been reported that in up to 5% of cases, it may have a fulminant course. A third will have a mild course without functional limitation. […] There is no evidence of effective treatment and only in transplant patients has an improvement or a detention in the progression of renal disease been achieved in the case of acute renal failure.

• #59

  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. […] The natural history of NSF is variable. As the condition progresses, the skin may take on a woody texture. Joint contractures or reduced range of movement are seen in 60 % of patients. Of 345 patients in the NSF Registry in 2009, almost 10 % were known to be restricted to a wheelchair. It is estimated that 5 % of patients have a rapidly progressive course, and NSF may increase mortality rates through restricted mobility and, more rarely, restricted ventilation. […] The clinical picture in NSF is very similar to scleromyxedema, but NSF favors the extremities and trunk rather than the head and neck. […] 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. No cases of complete remission have been described in the presence of continued renal impairment.

• #60 FDA Drug Safety Communication: New warnings for using gadolinium-based contrast agents in patients with kidney dysfunction | FDA

  https://www.fda.gov/drugs/drug-safety-and-availability/fda-drug-safety-communication-new-warnings-using-gadolinium-based-contrast-agents-patients-kidney

  The U.S. Food and Drug Administration (FDA) is requiring changes in the drug label for gadolinium-based contrast agents (GBCAs) to minimize the risk of nephrogenic systemic fibrosis (NSF), a rare, but serious, condition associated with the use of GBCAs in certain patients with kidney dysfunction. […] Symptoms of NSF include scaling, hardening and tightening of the skin; red or dark patches on the skin; and stiffness. NSF can also cause fibrosis of internal organs which may lead to death. There is no effective treatment for NSF. […] NSF has not been reported in patients with normal kidney function. […] If you have kidney disease, contact your healthcare professional right away if you experience any of the following after receiving a GBCA: burning, itching, swelling, scaling, hardening and tightening of the skin; red or dark patches on the skin; stiffness in joints with trouble moving, bending or straightening the arms, hands, legs or feet; pain in the hip bones or ribs; or muscle weakness.

• #61 FDA Drug Safety Communication: New warnings for using gadolinium-based contrast agents in patients with kidney dysfunction | FDA

  https://www.fda.gov/drugs/drug-safety-and-availability/fda-drug-safety-communication-new-warnings-using-gadolinium-based-contrast-agents-patients-kidney

  Advise patients with kidney disease to contact a healthcare professional if any of the following symptoms occurs after receiving a GBCA: burning, itching, swelling, scaling, hardening and tightening of the skin; red or dark patches on the skin; stiffness in joints with trouble moving, bending or straightening the arms, hands, legs or feet; pain in the hip bones or ribs; or muscle weakness.

• #62 Nephrogenic Systemic Fibrosis Lawsuit – AboutLawsuits.com

  https://www.aboutlawsuits.com/nephrogenic-systemic-fibrosis-781/

  It is primarily known to occur in those with chronic kidney problems or impaired renal function, who have undergone an MRI (magnetic resonance imaging) or MRA (magnetic resonance angiography), with a gadolinium contrast agent. […] Nephrogenic systemic fibrosis symptoms could include: […] Burning, itching, swelling of the skin […] Development of „thick skin” or „hard skin” which appears shiny […] Red or dark patches of skin […] Yellow spots on the whites of the eyes […] Stiff joints which cause trouble straightening or moving the limbs […] Deep pain in the hip bones or ribs […] Generalized muscle weakness. […] If symptoms of NSF appear after an MRI or MRA with contrast in an individual with impaired kidney function, a dermatologist will often recommend a punch biopsy to take a sampling of the skin so that a diagnosis of NSF can be determined.

• #63 Nephrogenic systemic fibrosis: the end of the story? – Malikova – Quantitative Imaging in Medicine and Surgery

  https://qims.amegroups.org/article/view/27779/html

  Unfortunately, clinical outcomes or follow-ups were reported only in 341 patients, 110 of them died; however, only in 4 of them their death was attributed directly to NSF. Partial resolution of NSF symptoms was found in 84 patients and even cure of NSF was reported in 12 patients; 40% of them all had acute kidney failure. Improvement of the disease was also reported in females during pregnancy (in 22 subjects) and in some patients after the cessation of -blockers (in 5 subjects). Approximately 18% of cases were reported as a stable disease. […] The second interesting finding is that NSF looks like an age dependent disease, because it was not reported in newborns and toddlers; the youngest reported child was 6 years old. This fact is remarkable, especially when newborns with immature kidneys were often examined with high doses of GBCA. Also, affection of the patients above 80 years were exceptional, only 7 cases were reported by Attari et al. […] Finally, and the most important, only seven NSF cases after GBCA exposures were reported since 2008. This result confirms the huge success of regulations, warnings and recommendations of authorities and radiology societies.

• #64 Nephrogenic Systemic Fibrosis | IJNRD

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

  Our research aimed to collect studies that reported data on the different clinical manifestations and their extent, as well as the different treatment options and their outcomes in patients of NSF. Hence, we report the common findings and highlight the efficacies of the most used treatment modalities. The commonly seen symptoms were cutaneous lesions (83.1%) and impaired mobility (74.7%). 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).

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