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Nephrogenic systemic fibrosis

Medically reviewed by Drugs.com. Last updated on Jun 6, 2023.

Overview

Nephrogenic systemic fibrosis is a rare disease that occurs mainly in people with advanced kidney failure with or without dialysis. Nephrogenic systemic fibrosis may resemble skin diseases, such as scleroderma and scleromyxedema, with thickening and darkening developing on large areas of the skin.

Nephrogenic systemic fibrosis can also affect internal organs, such as the heart and lungs, and it can cause a disabling shortening of muscles and tendons in the joints (joint contracture).

For some people with advanced kidney disease, being exposed to older gadolinium-based contrast agents (group 1) during magnetic resonance imaging (MRI) and other imaging studies has been identified as a trigger for development of this disease. Recognition of this link has dramatically reduced the incidence of nephrogenic systemic fibrosis. Newer gadolinium-based contrast agents (group 2) are not associated with an increased risk of systemic nephrogenic fibrosis.

Symptoms

Nephrogenic systemic fibrosis can begin days to months, and even years, after exposure to an older gadolinium-based contrast agent (group 1). Some signs and symptoms of nephrogenic systemic fibrosis may include:

In some people, involvement of muscles and body organs may cause:

The condition is generally long term (chronic), but some people may improve. In a few people, it can cause severe disability, even death.

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:

Risk factors

The highest risk of nephrogenic systemic fibrosis after exposure to older gadolinium-based contrast agents (group 1) occurs in people who:

Diagnosis

Diagnosis of nephrogenic systemic fibrosis is made with:

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:

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

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