Nephrogenic systemic fibrosis

Diagnosis

Diagnosis of nephrogenic systemic fibrosis is made with:

• Physical exam for signs and symptoms of the disease, and evaluation for a possible history of MRI using a gadolinium-based contrast agent when advanced kidney disease 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

here 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.

Certain treatments have shown limited success in some people with nephrogenic systemic fibrosis, but more research is needed to determine if these treatments help:

• Hemodialysis. In people with advanced chronic kidney disease who are receiving hemodialysis, performing hemodialysis immediately after receiving a gadolinium-based contrast agent 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.
• Extracorporeal photopheresis with ultraviolet A. 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.

These medications are experimental, but not currently in use. They have been shown to help some people, but side effects limit their use:

• Imatinib (Gleevec). Although this treatment shows some promise in reducing skin thickening and tightening, more research is needed.
• Pentoxifylline (Pentoxil). There is limited success with this medication, which theoretically decreases the thickness and stickiness (viscosity) of blood, aiding circulation. More research is needed.
• Sodium thiosulfate. Possible benefit has been shown using this medication, but more research is needed.
• High-dose intravenous immune globulin. Possible benefit has been shown using this medication, but more research is needed.