Nephrogenic systemic fibrosis (NSF) is a rare and serious condition that affects the skin, joints, and internal organs. It is primarily seen in people with advanced kidney disease or those who have recently undergone dialysis. The exact cause of NSF is not fully understood, but it is thought to be related to the exposure of certain contrast agents used during medical imaging studies, such as MRI scans.

Symptoms of NSF may include thickening and hardening of the skin, joint stiffness and pain, muscle weakness, and difficulty moving. These symptoms may develop gradually over time and may worsen with continued exposure to contrast agents.

Diagnosis of NSF may involve a physical exam, blood tests, imaging studies, and a biopsy of affected skin or other tissue. Treatment options for NSF are limited and may include managing symptoms through physical therapy, medications to reduce inflammation and pain, and in some cases, surgical removal of affected tissue.

Prevention of NSF involves avoiding exposure to certain types of contrast agents if you have advanced kidney disease or are undergoing dialysis. It is important to discuss any concerns or questions with your healthcare provider and to inform them of your medical history before undergoing any imaging studies.

While NSF is a serious and potentially life-threatening condition, it is rare and can often be prevented with appropriate precautions and monitoring. If you have kidney disease or are undergoing dialysis, it is important to work closely with your healthcare team to manage your condition and prevent potential complications.

The exact cause of nephrogenic systemic fibrosis isn't fully understood. Fibrous connective tissue forms in the skin and connective tissues, resulting in scarring of tissue throughout the body, most commonly the skin and subcutaneous tissues.

Exposure to older gadolinium-based contrast agents (group 1) during magnetic resonance imaging (MRI) has been identified as a trigger for development of this disease in people with kidney disease. This increased risk is thought to be related to the kidneys' reduced ability to remove the contrast agent from the bloodstream.

The Food and Drug Administration (FDA) recommends avoiding older gadolinium-based contrast agents (group 1) in people with acute kidney injury or chronic kidney disease.

Other conditions may increase the risk of nephrogenic systemic fibrosis when combined with existing kidney disease and exposure to older gadolinium-based contrast agents (group 1), but the link is uncertain. These include:

• Use of high-dose erythropoietin (EPO), a hormone that promotes the production of red blood cells, often used to treat anemia
• Recent vascular surgery
• Blood-clotting problems
• Severe infection

Avoidance of older gadolinium-based contrast agents (group 1) is key to preventing nephrogenic systemic fibrosis, as newer gadolinium-based contrast agents (group 2) are safer and are not associated with increased risk.

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.