Jan 8, 2024
Nephrogenic systemic fibrosis is a rare illness that mainly affects patients with advanced renal failure, whether or not they are receiving dialysis. Nephrogenic systemic fibrosis can cause notable skin patches to thicken and darken, similar to skin conditions like scleroderma and scleromyxedema.
Nephrogenic systemic fibrosis can also cause joint contracture, an incapacitating shortening of the muscles and tendons in the joints that can affect internal organs such as the heart and lungs.
There is a known risk factor for developing advanced kidney disease in some people (group 1) who have used older gadolinium-based contrast agents for imaging tests like magnetic resonance imaging (MRI).
Nephrogenic systemic fibrosis is significantly less common now that this link has been established. Modern contrast agents based on gadolinium (group 2) are not associated with an increased risk of systemic nephrogenic fibrosis.
The precise cause of nephrogenic systemic fibrosis is unknown. Fibrous connective tissue that forms in the skin and connective tissues causes tissue scarring throughout the body, but it most frequently occurs in the skin and subcutaneous tissues.
It has been discovered that older gadolinium-based contrast agents (group 1) used during magnetic resonance imaging (MRI) can cause renal dysfunction in persons with the illness. This increased risk is thought to be brought about by the kidneys' reduced ability to remove the contrast agent from the bloodstream.
The Food and Drug Administration (FDA) warns against using earlier gadolinium-based contrast agents (group 1) in patients with acute kidney injury or chronic kidney sickness.
It's unclear if additional conditions increase the risk of nephrogenic systemic fibrosis when combined with pre-existing kidney disease and exposure to prior gadolinium-based contrast agents (group 1). Among them are:
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Nephrogenic systemic fibrosis may appear days, weeks, months, or even years after exposure to an earlier group 1 gadolinium-based contrast agent. Some symptoms and indicators of nephrogenic systemic fibrosis include the following:
In certain people, muscle and organ involvement can lead to:
Although most cases of the illness are long-term (chronic), some people may get well. It can result in serious disability or even death in certain individuals.
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Nephrogenic systemic fibrosis is more common in individuals (group 1) exposed to older gadolinium-based contrast agents.
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A diagnosis of nephrogenic systemic fibrosis is made with:
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Nephrogenic systemic fibrosis has no known treatment, and no drug has ever been proven to consistently slow down or even reverse the disease's course. Given the rarity of nephrogenic systemic fibrosis, it may be difficult to perform a comprehensive study on this disorder.
Some patients with nephrogenic systemic fibrosis have responded quite well to a few treatments; however, more research is needed to determine whether these medications are helpful:
These medications are still experimental, despite not being used yet. Negative consequences limit their use, even if some have shown benefit from them:
Avoiding older gadolinium-based contrast agents (group 1) can help prevent nephrogenic systemic fibrosis, but newer gadolinium-based contrast agents (group 2) are safer and do not provide an increased risk.
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