Diagnosis

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

Treatment

There is no cure for nephrogenic systemic fibrosis, and no treatment is consistently successful in halting or reversing the progression of the disease. 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 people 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.
  • Extracorporeal photopheresis. This treatment involves drawing the blood outside the body and treating the blood with a drug that sensitizes it to ultraviolet light. The blood is then exposed to ultraviolet light and returned to the body. Some people have shown improvement after receiving this therapy.
  • Plasmapheresis. This procedure involves removing unwanted substances from the blood by separating solid blood cells from plasma, replacing plasma with donor plasma or albumin, then mixing it with the original solid blood cells and returning it to the body.

These medications have been shown to help some people, but side effects limit their use:

  • Pentoxifylline (Pentoxil). There is limited success with this medication, which theoretically decreases the thickness and stickiness (viscosity) of blood, aiding circulation.
  • Imatinib (Gleevec). 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.

Clinical trials

Explore Mayo Clinic studies testing new treatments, interventions and tests as a means to prevent, detect, treat or manage this disease.

Nephrogenic systemic fibrosis care at Mayo Clinic

June 16, 2016
References
  1. Kaewlai R, et al. Nephrogenic systemic fibrosis. American Journal of Roentgenology. 2012;199:W17.
  2. Galan A, et al. Nephrogenic systemic fibrosis. In: Treatment of Skin Disease: Comprehensive Therapeutic Strategies. 4th ed. Philadelphia, Pa.: Elsevier Limited; 2014. http://www.clinicalkey.com. Accessed March 9, 2016.
  3. Questions and answers on gadolinium-based contrast agents. U.S. Food and Drug Administration. http://www.fda.gov/Drugs/DrugSafety/DrugSafetyNewsletter/ucm142889.htm. Accessed March 9, 2016.
  4. Nephrogenic systemic fibrosis. National Organization for Rare Disorders. http://rarediseases.org/rare-diseases/nephrogenic-systemic-fibrosis/. Accessed March 9, 2016.
  5. Thomsen HS, et al. Nephrogenic systemic fibrosis and gadolinium-based contrast media: Updated ESUR Contrast Medium Safety Committee guidelines. European Radiology. 2013;23:307.
  6. Ferri FF. Nephrogenic systemic fibrosis. In: Ferri's Clinical Advisor 2016. Philadelphia, Pa.: Mosby Elsevier; 2016. https://www.clinicalkey.com. Accessed March 9, 2016.
  7. Miskulin D, et al. Nephrogenic systemic fibrosis/nephrogenic fibrosing dermopathy in advanced renal failure. http://www.uptodate.com/home. Accessed March 9, 2016.
  8. Leung N (expert opinion). Mayo Clinic, Rochester, Minn. March 15, 2016.