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Nephrogenic Systemic Fibrosis

Medically reviewed by Last updated on Oct 31, 2022.

What is nephrogenic systemic fibrosis?

Nephrogenic systemic fibrosis (NSF) is the thickening and hardening of your skin, mostly on your arms and legs. Your muscles, joints, organs, and the tissue that covers your brain may also be affected. It is a condition normally found in people with kidney disease.

What increases my risk for NSF?

The cause of NSF may not be known. Any of the following can increase your risk:

  • Contrast dye called gadolinium that is used in medical tests
  • Medical conditions, such as kidney disease, hepatitis, or pulmonary fibrosis
  • High levels of iron, copper, or zinc in blood or tissues
  • Medicine that cause lactic acidosis
  • Vascular surgery to repair your blood vessels

What are the signs and symptoms of NSF?

  • Painful or itchy skin
  • Reddish bumps on your skin
  • Thick, hard, or dimpled skin, like an orange peel
  • Yellow spots or redness on the whites of your eyes
  • Hair loss
  • Swelling of your hands and feet, and pain and muscle weakness in your arms or legs
  • Chest pain

How is NSF diagnosed?

  • Blood tests may be needed to check your kidney function.
  • A skin biopsy is when a sample of your skin is taken and sent to a lab for tests.

How is NSF treated?

The goal of treatment is to improve your kidney function, decrease your pain, and improve your joint movement. You may need any of the following:

  • Prescription pain medicine may be given. Ask your healthcare provider how to safely take this medicine.
  • Dialysis cleans your blood when your kidneys cannot. Extra water, chemicals, and waste products are removed from your blood by a dialyzer or dialysis machine. The dialysis machine does this by passing your blood through a filter, then returning it back to you. Ask for more information about dialysis.
  • Photopheresis is when your blood is removed and treated with ultraviolet (UV) light and medicine. Blood is then returned to your body. Ask for more information about photopheresis.

Treatment options

The following list of medications are in some way related to or used in the treatment of this condition.

How can I manage my symptoms?

  • Go to physical therapy. A physical therapist teaches you exercises to help improve movement and strength. Physical therapy can help loosen your tight skin and prevent contractures. A contracture is when your skin or muscles tighten, causing problems with how you move your joints.
  • Go to the pain clinic. Healthcare providers at a pain clinic can help you learn new ways to control your pain.

When should I contact my healthcare provider?

  • Your arms or legs are swollen, or you have trouble moving them.
  • Your skin becomes red or painful.
  • You pain is worse, even after you take medicine.
  • You have questions or concerns about your condition or care.

When should I seek immediate care or call 911?

  • You have severe pain.
  • You suddenly have trouble breathing.

Care Agreement

You have the right to help plan your care. Learn about your health condition and how it may be treated. Discuss treatment options with your healthcare providers to decide what care you want to receive. You always have the right to refuse treatment. The above information is an educational aid only. It is not intended as medical advice for individual conditions or treatments. Talk to your doctor, nurse or pharmacist before following any medical regimen to see if it is safe and effective for you.

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