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.

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.

Last Reviewed:
October 10, 2016
Last Updated:
August 09, 2017
Content Source: