Nephrogenic systemic fibrosis is a rare disease that occurs mainly in people with advanced acute kidney failure or those who are receiving dialysis. The disease causes extensive patches of thickened and hardened skin. Nephrogenic systemic fibrosis can also affect internal organs, such as the heart, kidneys 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 gadolinium-containing contrast agents during magnetic resonance imaging (MRI) has been identified as a trigger for development of this disease. Recognition of this link has dramatically reduced the incidence of nephrogenic systemic fibrosis.

  • 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.

About

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

Symptoms

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 #1 for kidney disorders in the U.S. News & World Report Best Hospitals rankings. Mayo Clinic in Scottsdale, Ariz., is ranked among the Best Hospitals for kidney disorders, and Mayo Clinic in Jacksonville, Fla., is ranked high performing for kidney disorders by U.S. News & World Report.

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Diagnosis of nephrogenic systemic fibrosis is made by:

  • Physical exam for signs and symptoms of the disease, and evaluation for a possible history of MRI using gadolinium when advanced kidney failure is present
  • A sample of tissue (biopsy) taken from the skin and muscle
  • Other tests as needed that may indicate involvement of muscles and internal organs

There is no cure for nephrogenic systemic fibrosis, and no treatment is consistently successful in halting or reversing the progression of the disease. The lesions may be more treatable earlier in the disease course, but less treatable after muscles are affected. Nephrogenic systemic fibrosis only occurs rarely, making it difficult to conduct large studies.

Mayo Clinic doctors have experience with the following treatments for nephrogenic systemic fibrosis. More research is needed to determine if these treatments help, but they have shown limited success in some people.

  • Hemodialysis. In patients with severely reduced kidney function, performing hemodialysis immediately after receiving gadolinium-containing contrast may decrease the possibility of nephrogenic systemic fibrosis.
  • Physical therapy. Physical therapy that helps stretch the involved limbs may help slow the progression of joint contractures and preserve movement.
  • Kidney transplant. For people who are appropriate candidates, improvement in renal function because of a kidney transplant may help improve nephrogenic systemic fibrosis over time.
  • Ultraviolet A phototherapy. Exposure of the skin to ultraviolet A light may reduce the skin thickening and hardening, but it's unclear if the treatment penetrates deeply enough into the skin to be effective.
  • Reduced erythropoietin (EPO) dosage. Reducing the doses of EPO can help some people who have nephrogenic systemic fibrosis.

These medications have been shown to help some people, but their side effects limit their use. More research is needed to determine if these options help:

  • Topical cream. Some people report improvement from calcipotriene in early stages of the disease.
  • Oral or topical steroids. These medications have helped some people, but due to their side effects, their use is largely discouraged.
  • Thalidomide. Some people improve while taking this drug, but its long-term side effects may be an issue.
  • Hydroxychloroquine. Some people benefit from this drug, but side effects can affect the eyes, and it must be closely monitored.
  • Minocycline. Some people have reported benefit from this medication, but it can affect the intestinal tract and lead to fungal infections and sun sensitivity.
  • Pentoxifylline. There is limited success with this medication, which theoretically decreases the thickness and stickiness (viscosity) of blood, aiding circulation.
  • Imatinib. Although this treatment shows some promise in reducing skin thickening and tightening, more research is needed.
  • Sodium thiosulfate. Possible benefit has been shown using this medication, but more research is needed.

Mayo Clinic works with hundreds of insurance companies and is an in-network provider for millions of people. In most cases, Mayo Clinic doesn't require a physician referral. Some insurers require referrals or may have additional requirements for certain medical care. All appointments are prioritized on the basis of medical need.

Specialists in dermatology and nephrology and hypertension care for adults who have nephrogenic systemic fibrosis.

For appointments or more information, call the Central Appointment Office at 800-446-2279 (toll-free) 8 a.m. to 5 p.m. Mountain Standard Time, Monday through Friday or complete an online appointment request form.

Specialists in dermatology and nephrology and hypertension care for adults who have nephrogenic systemic fibrosis.

For appointments or more information, call the Central Appointment Office at 904-953-0853 8 a.m. to 5 p.m. Eastern time, Monday through Friday or complete an online appointment request form.

Specialists in dermatology and nephrology and hypertension care for children and adults who have nephrogenic systemic fibrosis.

For appointments or more information, call the Central Appointment Office at 507-538-3270 7 a.m. to 6 p.m. Central time, Monday through Friday or complete an online appointment request form.

See information on patient services at the three Mayo Clinic locations, including transportation options and lodging.

Mayo Clinic researchers identified a link between nephrogenic systemic fibrosis and high-dose erythropoietin administration in some people.

Mayo publications

See a list of publications by Mayo Clinic doctors on nephrogenic systemic fibrosis on PubMed, a service of the National Library of Medicine.

Jun. 17, 2013