Causes

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