Nephrogenic Systemic Fibrosis

What is Nephrogenic Systemic Fibrosis?

Nephrogenic Systemic Fibrosis is fairly rare and it is a condition that primarily occurs in those who have advanced kidney failure.  It can occur whether they have had dialysis or not.  It is similar to some skin diseases like scleromyxedema and scleroderma as the main characteristics are the darkening or thickening of large patches of skin.

It can affect the internal organs and cause the shortening of tendons and muscles. For some people, the exposure to some gadolinium contrast agents from MRIs or other imaging tests has triggered nephrogenic systemic fibrosis.

What are the Symptoms of Nephrogenic Systemic Fibrosis?

Nephrogenic systemic fibrosis progresses quickly after it begins.  Signs of this disease include patches of skin (usually on the arms or legs but can occur on other parts of the body, too) that become thick or hard and has a darker than normal pigmentation. The skin may swell and feel tight and there can be sharp pain, itching, or burning.  It can even inhibit movement and there can be a loss of flexibility. Some people even get ulcers or blisters in the affected areas.

If the body organs or muscles are involved, there can be bone pain, limited motion of affected limbs, weakness of the muscles, problems with the function of some organs, and blood clots.

Nephrogenic Systemic Fibrosis Causes

The causes of Nephrogenic Systemic Fibrosis (NSF) are not fully understood although the exposure to gadolinium, a contrast material used in the magnetic resonance imaging (MRI), is often contributed to the development of this disease. The link between gadolinium and NSF is so great that the FDA required gadolinium-based contrast agents, or GBCA, to carry the warning labels about the risk of NSF to patients with renal diseases. In addition, the FDA also stated that the three gadolinium contrasting agents of Ominscan, Magnevist, and Optimark are linked to NSF.

The fact that gadolinium is the only cause of the disease is not definite or certain. There are reports of patients who develop this disease without being exposed to gadolinium. Other causes that may have triggered NSF include recent vascular surgery, blood-clotting problems, and severe infection. What is also considered to be the possible cause of NSF is the use of the high dosage of erythropoietin (EPO), a hormone that helps produce red blood cells used to fight anemia.

How is Nephrogenic Systemic Fibrosis Treated?

Nephrogenic fibrosis is a long term condition and none of the treatments are always successful.

Treatment is focused on reversing progress of the condition or stopping progress.  Here are some of the treatments that doctors have had success with:

  • Physical therapy: stretching of the limbs can slow the progression of the disease and make movement easier
  • Ultraviolet A phototherapy: skin thickening and hardening can be reduced when the skin is exposed to ultraviolet A light.
  • Hemodialysis: if hemodialysis is performed right after a gladolinium-containing agent is received, it can reduce the chance of having nephrogenic systemic fibrosis.
  • Extracorporeal phopheresis means that blood is removed from the body and then sensitized to ultraviolet light. The blood is then exposed to ultraviolet light and returned to the body.
  • Kidney transplant:
  • Plasmapheresis: bad foods are and unwanted substances are removed from the bloodstream by swapping out unwanted substances.

Some medications can be helpful, too.  Pentoxifylline (Pentoxil) helps with the circulation of the blood by making it less thick and sticky. Sodium thiosulfate is a medication that is currently under research and some possible benefits have been found. Imatinib (Gleevec) is also being researched because it has been found to have some results for reducing the tightening and thickening of the skin.

Nephrogenic Systemic Fibrosis Prevention

It is up to physicians to prevent NSF from forming in patients with kidney diseases. They should follow the FDA’s recommendation of not using the MRI scans that contain gadolinium. Instead, they should try to use alternative imaging modalities that do not involve the gadolinium-based contrast agents. If it is not possible for the providers to obtain the essential diagnostic information with the noncontrast-enhanced MRI, they can use the MRI with GBCA. Even then they should try to use it judiciously to avoid putting the patients at risk.

Providers should also avoid the use of Ominscan, Magnevist, and Optimark when performing MRIs on patients with advanced stages of the chronic kidney diseases per the FDA’s recommendation. Healthcare professionals are recommended to administer hemodialysis to patients after they receive the gadolinium-containing MRIs to prevent NSF from developing; however, it has not been proven to be effective so far.

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Last Reviewed:
October 10, 2016
Last Updated:
January 25, 2018