- Teamwork. Typically, a kidney specialist (nephrologist) and a skin disease specialist (dermatologist) work together to treat nephrogenic systemic fibrosis. But because this disease can affect many different body systems, Mayo Clinic's multidisciplinary approach allows quick consultation with other specialists as needed.
- Expertise and experience. Even though nephrogenic systemic fibrosis is uncommon, Mayo Clinic doctors have the expertise and experience to diagnose and treat this disease. Radiologists at Mayo Clinic are skilled at finding alternatives to gadolinium-containing contrast agents in at-risk people needing imaging tests.
Nephrogenic systemic fibrosis may resemble skin diseases, such as scleroderma and scleromyxedema, with thickening and darkening developing on large areas of the skin.
The Food and Drug Administration (FDA) recommends not using gadolinium-containing contrast agents in people with acute kidney injury or chronic kidney disease. The highest risk of nephrogenic systemic fibrosis after gadolinium exposure 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
This increased risk is thought to be related to the reduced ability of these people's kidneys to remove the contrast agent from the bloodstream. Prolonged exposure to high levels of gadolinium may possibly trigger nephrogenic systemic fibrosis.
Nephrogenic systemic fibrosis can begin days to months after exposure to gadolinium-containing contrast, but progresses quickly. The condition is generally long term (chronic), but some patients may improve. In a few people, it can cause severe disability, even death. The disease occurs in people of all ages, but usually strikes in middle age.
Although more research is needed, possible conditions that may lead to or promote the disease when severe kidney disease and exposure to gadolinium-containing contrast are present include:
- Use of erythropoietin (EPO), a hormone that promotes the production of red blood cells, often used to treat anemia
- Blood vessel injury
- Blood-clotting problems
- Severe infection
Some signs and symptoms of nephrogenic systemic fibrosis include:
- Swelling and tightening of the skin
- Thickening and hardening of the skin, typically on the arms and legs and sometimes on the body, but almost never the face or head
- Skin that may feel "woody" and develop an orange peel appearance and darkening (excess pigmentation)
- 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
- Reduced internal organ function, including heart, lung, liver or kidney
- Yellow plaques on the white surface (sclera) of the eyes
- Calcium deposits in tissue and muscle
- Blood clots
Nephrogenic systemic fibrosis is also associated with failure of kidney transplant.
Mayo Clinic in Rochester, Minn., ranks No. 1 for kidney disorders in the U.S. News & World Report Best Hospitals rankings. Mayo Clinic in Scottsdale, Ariz., ranks among the Best Hospitals for kidney disorders by U.S. News & World Report. Mayo Clinic in Jacksonville, Fla., is ranked high performing for kidney disorders by U.S. News & World Report.
June 17, 2013