Nephrogenic Systemic Fibrosis

Treatment

There is no cure for nephrogenic systemic fibrosis, and no treatment is consistently successful in halting or reversing the progression of the disease. Some medications can help some patients, but many have serious side effects. The lesions may be more treatable earlier in the disease course, less treatable after muscles are affected.

Mayo Clinic doctors have experience with the following treatments for nephrogenic systemic fibrosis and continue to investigate these and other options.

• Reduced erythropoietin (EPO) dosage. Many people with kidney disease have low levels of red blood cells (anemia). Erythropoietin (epoetin alfa), a hormone that promotes the production of red blood cells, is often used to treat anemia. Reducing the doses of EPO can help some people who have nephrogenic systemic fibrosis.
• Oral steroids (prednisone). These medications have helped some people, but due to their side effects, their use is largely discouraged.
• Topical cream. Some people report improvement from calcipotriene (Dovonex) in early stages of the disease.
• Photopheresis. Your blood is removed, exposed to ultraviolet radiation and then returned to you.
• Plasmapheresis. Removing the liquid portion of your blood (plasma) has been shown to improve the disease in some people with dual liver-kidney transplants.
• Thalidomide (Thalomid). Some people improve while taking this drug, but its long-term side effects may be an issue.
• Hydroxychloroquine (Plaquenil). Some people benefit from this drug, but side effects can affect the eyes, and it must be closely monitored.
• Minocycline (Solodyn). Some people have reported benefit from this medication, but it can affect the intestinal tract and lead to fungal infections and sun sensitivity.
• Pentoxifylline (Trental). There are some reports of success with this medication, which theoretically decreases the viscosity of blood and aids circulation and can have antioxidant effects.
• Physical therapy. Physical therapy, particularly swimming, may help slow the progression of joint problems.
• Kidney transplant. Some people have complete resolution of the disease following kidney transplant. Mayo researchers followed 11 people with nephrogenic systemic fibrosis who received a kidney transplant and compared the results with a similar group of six people who received dialysis treatment. Overall, they found no difference in symptom relief between the two groups.