Nephrogenic systemic fibrosis is a rare disease that occurs mainly in people with advanced kidney failure with or without dialysis. Nephrogenic systemic fibrosis may resemble skin diseases, such as scleroderma and scleromyxedema, with thickening and darkening developing on large areas of the skin.
Nephrogenic systemic fibrosis can also affect internal organs, such as the heart and lungs, and it can cause a disabling shortening of muscles and tendons in the joints (joint contracture).
For some people with advanced kidney disease, being exposed to older gadolinium-based contrast agents (group 1) during magnetic resonance imaging (MRI) and other imaging studies has been identified as a trigger for development of this disease. Recognition of this link has dramatically reduced the incidence of nephrogenic systemic fibrosis. Newer gadolinium-based contrast agents (group 2) are not associated with an increased risk of systemic nephrogenic fibrosis.
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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:
- Swelling and tightening of the skin
- 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
- Skin thickening that inhibits movement, resulting in loss of joint flexibility
- Rarely, blisters or ulcers
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
The condition is generally long term (chronic), but some people may improve. In a few people, it can cause severe disability, even death.
The exact cause of nephrogenic systemic fibrosis isn't fully understood. Fibrous connective tissue forms in the skin and connective tissues, resulting in scarring of tissue throughout the body, most commonly the skin and subcutaneous tissues.
Exposure to older gadolinium-based contrast agents (group 1) during magnetic resonance imaging (MRI) has been identified as a trigger for development of this disease in people with kidney disease. This increased risk is thought to be related to the kidneys' reduced ability to remove the contrast agent from the bloodstream.
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.
Other conditions may increase the risk of nephrogenic systemic fibrosis when combined with existing kidney disease and exposure to older gadolinium-based contrast agents (group 1), but the link is uncertain. These include:
- Use of high-dose erythropoietin (EPO), a hormone that promotes the production of red blood cells, often used to treat anemia
- Recent vascular surgery
- Blood-clotting problems
- Severe infection
The highest risk of nephrogenic systemic fibrosis after exposure to older gadolinium-based contrast agents (group 1) occurs in people who:
- Have moderate to severe kidney disease
- Have had a kidney transplant but have compromised renal function
- Are receiving hemodialysis or peritoneal dialysis
- Have acute kidney injury
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.