Is nephrogenic systemic fibrosis curable?
here is no cure for nephrogenic systemic fibrosis, and no treatment is consistently successful in halting or reversing the progression of the disease.
What does nephrogenic systemic fibrosis look like?
Reddened or darkened patches on the skin. Thickening and hardening of the skin, typically on the arms and legs and sometimes on the body, but almost never on the face or head. Skin that may feel "woody" and develop an orange-peel appearance. Burning, itching or severe sharp pains in areas of involvement.
How many people have nephrogenic systemic fibrosis?
NSF is a rare condition, with over 335 cases identified in the NSF Registry. [7] NSF affects males and females in approximately equal numbers.
Does dialysis prevent nephrogenic systemic fibrosis?
How is nephrogenic systemic fibrosis treated? Currently, there are no effective treatments. Dialysis can remove gadolinium from the body after an MRI, but there is no evidence that this stops patients from developing nephrogenic systemic fibrosis.
What does gadolinium do to the brain?
Gadolinium enhances the quality of MRI by altering the magnetic properties of water molecules that are nearby in the body. Gadolinium can improve the visibility of specific organs, blood vessels, or tissues and is used to detect and characterize disruptions in normal physiology.
What treatment does nephrogenic systemic patient get?
Of all treatments, extracorporeal photopheresis (ECP) seems to provide the best, albeit mild and extremely expensive, treatment modality for nephrogenic systemic fibrosis. In one patient, ECP resulted in some improvement in skin texture.
Does dialysis prevent NSF?
Approximately 98% is eliminated after three consecutive dialysis sessions (Joffe 1998; Tombach 2002; Gheuens 2014). Based on these data, early haemodialysis would be an effective treatment for preventing NSF.
What causes nephrogenic systemic fibrosis?
Nephrogenic systemic fibrosis (NSF) is a rare disorder that occurs in some individuals with reduced kidney function, who have been exposed to an intravenous contrast material that contains gadolinium. A contrast material is a dye that is sometimes used during magnetic resonance imaging (MRI).
Can gadolinium cause blood clots?
It can cause a shortening of muscles and tendons causing muscle weakness, severe pain, limited mobility, reduced organ function, and blood clots. Patient advocacy groups have reported severe disease in patients who received Gadolinium-based contrast agents during MRI procedures.
Does gadolinium cause kidney damage?
Objectives: Gadolinium-based contrast media (Gd-CM) are reported to induce acute kidney injury (AKI) in a high-risk population group at the usual dose for magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) examinations.
How do you get gadolinium out of your system?
Chelation is a process where doctors administer chelating agents to patients. These agents bind gadolinium and remove it from the body through the kidneys. Health providers may administer chelating agents through an IV, with a pill, as a suppository under the tongue or through a rectal suppository.
What are the symptoms of NSF?
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.
What are the symptoms of gadolinium toxicity?
ToxicityPain in the bones or joints.Burning or “pins and needle” sensations in the skin.Brain fog.Headache.Vision or hearing changes.Changes to the skin, such as thickening or discoloration.Nausea, vomiting, or diarrhea.Difficulty breathing.More items...
How long does gadolinium stay in the body?
With normal kidney function, most of the gadolinium is removed from your body in the urine within 24 hours.
Are NSF and NFD the same disease?
Nephrogenic systemic fibrosis (NSF), also known as nephrogenic fibrosing dermopathy (NFD), is a disease of fibrosis of the skin and internal organs reminiscent but distinct from scleroderma or scleromyxedema.
Is gadolinium hard on the kidneys?
Newer contrast agents that contain gadolinium are generally safe for people with kidney disease, even those who receive dialysis. But that might not be the case with older contrast agents. The type of gadolinium used in older contrast agents isn't safe for people with moderate or advanced chronic kidney disease.
How long does it take for nephrogenic systemic fibrosis to develop?
Patients can develop symptoms of nephrogenic systemic fibrosis as early as the day they are exposed to gadolinium-based contrast agents, or up to several months later. In one study, 50% of patients had symptoms within 12 days after exposure.
What is the chemical element that causes nephronic systemic fibrosis?
The rare condition nephronic systemic fibrosis, which affects the skin and organs, has been seen in a small number of kidney patients after exposure to a chemical element called gadolinium.
What is NSF in medical terms?
Nephrogenic systemic fibrosis (NSF) is a rare disease that can affect different parts of the body. It most commonly first appears as swelling or itching of the skin. It can lead to thickening or hardening of the skin and deposits in other parts of the body. The disease was first observed in 1997, and was thought to only affect the skin.
Can a doctor order a biopsy for nephrogenic systemic fibrosis?
The only way to be certain is to have a deep skin biopsy (where tissue is taken for microscopic examination). A doctor would most likely only order such a test if he or she had reason to believe the patient may have nephrogenic systemic fibrosis.
Is gadolinium safe for renal failure?
This finding reflects the use of more stable, less toxic gadolinium agents and limiting the use of gadolinium in patients with renal failure. Agents that are macrocytic are much less toxic. As a result, the safe use of gadolinium and avoidance of fibrosis has increased.
Does gadolinium cause nephrogenic systemic fibrosis?
Exactly how gadolinium leads to nephrogenic systemic fibrosis is unclear. However, it does appear that the risk of developing the condition goes up as a person is exposed to certain types called linear gadolinium and as a higher amount of gadolinium is given. Since 2009, no new cases of NSF have been reported.
Can nephrogenic systemic fibrosis be fatal?
Nephrogenic systemic fibrosis may be a progressive condition. As it gets worse, it can go well beyond the skin and cause multiple organs to fail. It can be fatal.
What is nephrogenic systemic fibrosis?
Nephrogenic systemic fibrosis. Nephrogenic systemic fibrosis is a rare syndrome that involves fibrosis of 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. Epidemiological studies suggest that the incidence ...
What is the risk factor for kidney failure?
Risk factors. impaired kidney function is the major risk factor. Nephrogenic systemic fibrosis is a rare syndrome that involves fibrosis of 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.
What is the term for fibrosing skin?
This condition was originally termed “nephrogenic fibrosing dermopathy” as initially only skin involvement in patients with impaired kidney function was observed, and later renamed “nephrogenic systemic fibrosis” to better describe its systemic nature. The term "gadolinium-associated systemic fibrosis" has also been proposed to reflect the fact that impaired kidney function is not in itself the cause of NSF.
What are the differential diagnoses for NSF?
The differential diagnoses for NSF include diffuse cutaneous or limited cutaneous systemic sclerosis, scleromyxedema, lipodermatosclerosis, scleroedema diabeticorum, graft versus host disease, eosinophilic fasciitis; eosinophilia-myalgia syndrome; porphyria cutanea tarda, and other disorders. The nearly universal absence of facial skin involvement in NSF, presence of yellow plaques on the sclera of the eyes, absence of Raynaud’s phenomenon, and other differences in presentation can aid the proper diagnosis. History of exposure to GBCAs would favor NSF as the differential diagnosis.
What is NSF in medical terms?
NSF is an iatrogenic disease caused by exposure to gadolinium-based contrast agents used in magnetic resonance imaging.
How long does it take for NSF to develop?
Clinical features of NSF develop within days to months following exposure to GBCA. 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.
Is dialysis a preventative measure for NSF?
The only known measure for prevention of NSF is the non-use or cautious use of GBCAs in patients with renal impairment, including preferential use of safer, macrocyclic GBCAs. Performing dialysis immediately after the MRI exam is recommended for patients already in dialysis treatment, but there is no evidence for introducing dialysis in non-dialytic patients for prevention of NSF. Screening for impaired kidney function is routinely conducted and has drastically reduced the incidence of NSF.
What are the risk factors for NSF?
An additional critical risk factor for the development of NSF is renal impairment. All GBCAs are cleared, at least in part, from the body by the kidneys, and almost all cases of NSF have occurred in individuals with advanced kidney disease (eGFR <30 mL/min/1.73m2). However, other patient-level risk factors have been proposed as well, including the severity and chronicity of kidney dysfunction and inflammation.
What is NSF in MRI?
Nephrogenic systemic fibrosis (NSF) is a debilitating and , in most cases, fatal condition associated with exposure to certain gadolinium-based contrast agents (GBCA) administered during magnetic resonance imaging (MRI) or angiography (MRA) scans. Clinically, NSF presents as fibrosis of the skin and internal organs such as the heart, liver, ...
What is the pathogenesis of NSF?
The pathogenesis of NSF is believed to begin with the displacement of the Gd ion from its chelate by another metallic cation (Fe +3, Zn +2, or Ca +2) through a so-called transmetalation reaction :
What is NSF in medical terms?
Nephrogenic systemic fibrosis (NSF) is a rare, progressive, usually fatal disease characterized by skin thickening, painful joint contractures, and fibrosis of multiple organs including the lungs, liver, muscles, and heart .
How long does it take for NSF to develop?
NSF usually develops clinically within days to months following gadolinium exposure, although rare cases have been reported years later. Nearly all patients have been in severe renal failure, and many were on dialysis. To my knowledge only one case has occurred in a patient with a GFR slightly greater than 30 mL/min/1.73m².
What are the causes of renal insufficiency?
The strong association with renal insufficiency most likely relates to the prolonged biological half-life due to prolonged excretion of gadolinium. However, other factors have been imputed, including metabolic acidosis; elevated iron and phosphate levels; erythropoietin therapy; vasculopathy; and infectious/inflammatory mediators.
Is NSF eliminated?
Today, NSF has been completely eliminated due to these measures. In more recent times, however, gadolinium-induced plaques have been reported in the extremities not meeting the full criteria for NSF. The story of NSF is sad one that we radiologists created.
What causes nephrogenic systemic fibrosis?
The cause of nephrogenic systemic fibrosis is attributed to the connexation of renal insufficiency and gadolinium exposure from imaging studies . The exact degree of renal insufficiency that sets up the development of nephrogenic systemic fibrosis is not known. Risk factors include advanced chronic kidney disease (stages 3, ...
What organs are affected by fibrosis?
Loss of flexibility and severe limitations in movement at the joints of the ankles, knees, feet, arms, wrists, and hands; Fibrosis involving the internal organs, including the lungs (with reduced diffusing capacity for carbon monoxide) and diaphragm (with respiratory failure), myocardium, pericardium and pleura.
What is the NSF?
Nephrogenic Systemic Fibrosis (NSF) is a rare disease involving severe thickening and hardening of the skin (fibrosis) overlying the extremities and trunk. The cause of nephrogenic systemic fibrosis is attributed to the connexation of renal insufficiency and gadolinium exposure from imaging studies. ...
What is the diagnostic test for NSF?
Diagnostic testing: Diagnostic testing may include positive antinuclear antibody tests and the presence of hepatitis B or C. All people affected with NSF have a history of renal insufficiency of varying severity and duration and gadolinium exposure. A small number of individuals have primarily liver disease.
What are the symptoms of NSF?
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. Fibrosis (thickening) involving the internal organs, including the lungs (with reduced diffusing capacity for carbon monoxide) and diaphragm (with respiratory failure), myocardium, pericardium and pleura has been reported. NSF affects males and females in approximately equal numbers. NSF may occur in children but most commonly affects the middle-aged.
What are the physical symptoms of NSF?
Physical findings: Symptoms of NSF include: Loss of flexibility and severe limitations in movement at the joints of the ankles, knees, feet, arms, wrists, and hands;
Is NSF a debilitating disease?
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.
Overview
Nephrogenic systemic fibrosis is a rare syndrome that involves fibrosis of 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. Epidemiological studies suggest that the incidence of NSF is unrelated to gender or ethnicity and it is not thought to have a genetic basis. After GBCAs were identified as a cause of the disorder in 2006, and screening and prevention m…
Signs and symptoms
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 fing…
Causes
NSF is an iatrogenic disease caused by exposure to gadolinium-based contrast agents used in magnetic resonance imaging.
Risk factors
Impaired kidney function reduces the clearance of GBCAs and is the major risk factor for the development of NSF. The etiology or duration of renal failure seems not to be relevant, but NSF risk greatly depends on the residual kidney function. The majority of NSF cases have been identified in patients with stage 5 CKD, but NSF has also developed in patients with stage 4 and 3 CKD, and those with acute kidney injury, even if kidney function subsequently returned to normal …
Mechanism
De-chelation of Gd(III) is responsible for the toxicity associated with gadolinium complexes such as GBCAs, and the toxicity appears to be a consequence of Zn , Cu , and Ca transmetallation in vivo. This hypothesis is supported by acute toxicity experiments, which demonstrate that despite a 50-fold range of LDse values for four Gd(III) complexes, all become lethally toxic when they release precisely the same quantity of Gd(III). It is also supported by subchronic rodent toxicity e…
Diagnosis
There is no specific imaging finding for NSF, and the diagnosis is a clinicopathological one, based on presentation and histological findings.
At the microscopic level, NSF shows a proliferation of dermal fibroblasts and dendritic cells, thickened collagen bundles, increased elastic fibers, and deposits of mucin. More recent case reports have described the presence of sclerotic bodies (also known as elastocollagenous balls…
Prevention
The only known measure for prevention of NSF is the non-use or cautious use of GBCAs in patients with renal impairment, including preferential use of safer, macrocyclic GBCAs. Performing dialysis immediately after the MRI exam is recommended for patients already in dialysis treatment, but there is no evidence for introducing dialysis in non-dialytic patients for prevention of NSF. Screening for impaired kidney function is routinely conducted and has drasti…
Treatment
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.