Nephrogenic Systemic Fibrosis: Causes, Symptoms and Risk Factors

Nephrogenic systemic fibrosis is a rare illness that mainly affects patients with advanced renal failure, whether or not they are receiving dialysis. Nephrogenic systemic fibrosis can cause notable skin patches to thicken and darken, similar to skin conditions like scleroderma and scleromyxedema.

Nephrogenic systemic fibrosis can also cause joint contracture, an incapacitating shortening of the muscles and tendons in the joints that can affect internal organs such as the heart and lungs.

There is a known risk factor for developing advanced kidney disease in some people (group 1) who have used older gadolinium-based contrast agents for imaging tests like magnetic resonance imaging (MRI). 

Nephrogenic systemic fibrosis is significantly less common now that this link has been established. Modern contrast agents based on gadolinium (group 2) are not associated with an increased risk of systemic nephrogenic fibrosis.

Causes Of Nephrogenic Systemic Fibrosis

The precise cause of nephrogenic systemic fibrosis is unknown. Fibrous connective tissue that forms in the skin and connective tissues causes tissue scarring throughout the body, but it most frequently occurs in the skin and subcutaneous tissues.

It has been discovered that older gadolinium-based contrast agents (group 1) used during magnetic resonance imaging (MRI) can cause renal dysfunction in persons with the illness. This increased risk is thought to be brought about by the kidneys' reduced ability to remove the contrast agent from the bloodstream.

The Food and Drug Administration (FDA) warns against using earlier gadolinium-based contrast agents (group 1) in patients with acute kidney injury or chronic kidney sickness.

It's unclear if additional conditions increase the risk of nephrogenic systemic fibrosis when combined with pre-existing kidney disease and exposure to prior gadolinium-based contrast agents (group 1). Among them are:

• One popular treatment for anemia is high-dose erythropoietin (EPO), a hormone that promotes the production of red blood cells.
• Recent vascular surgery
• Clotting blood 
• Severe infection

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Symptoms Of Nephrogenic Systemic Fibrosis

Nephrogenic systemic fibrosis may appear days, weeks, months, or even years after exposure to an earlier group 1 gadolinium-based contrast agent. Some symptoms and indicators of nephrogenic systemic fibrosis include the following:

• Tightness and expansion of the skin
• Reddish or dark patches on the skin
• There may be scorching, itching, or excruciatingly sharp pains in the affected places; the skin may feel "woody" and take on an orange-peel appearance; these symptoms are typically found on the arms, legs, and seldom other parts of the body.
• Skin thickening that decreases joint flexibility and restricts range of motion
• Ulcers or blisters are not common.

In certain people, muscle and organ involvement can lead to:

• Weakened muscles
• Restriction of joint motion brought on by contractures in the arms, hands, legs, and feet
• Bone discomfort, especially in the ribs or hip bones
• Diminished function of internal organs, such as the liver, heart, lungs, diaphragm, or gastrointestinal tract
• Patches of yellow color on the eyes' white sclera

Although most cases of the illness are long-term (chronic), some people may get well. It can result in serious disability or even death in certain individuals.

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Risk Factors Of Nephrogenic Systemic Fibrosis

Nephrogenic systemic fibrosis is more common in individuals (group 1) exposed to older gadolinium-based contrast agents.

• Have a moderate to severe form of renal illness.
• After receiving a kidney transplant, have compromised renal function and are receiving hemodialysis or peritoneal dialysis
• Have substantial kidney damage

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Diagnosis Of Nephrogenic Systemic Fibrosis

A diagnosis of nephrogenic systemic fibrosis is made with:

• In cases of advanced kidney disease, a gadolinium-based contrast agent should be used during a physical examination to assess any potential MRI history and search for disease signs and symptoms.
• A biopsy, or tissue sample, taken from the skin and muscles
• If more testing is required, it can show that internal organs and muscles are involved.

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Treatment Of Nephrogenic Systemic Fibrosis

Nephrogenic systemic fibrosis has no known treatment, and no drug has ever been proven to consistently slow down or even reverse the disease's course. Given the rarity of nephrogenic systemic fibrosis, it may be difficult to perform a comprehensive study on this disorder.

Some patients with nephrogenic systemic fibrosis have responded quite well to a few treatments; however, more research is needed to determine whether these medications are helpful:

• Hemodialysis: Patients on hemodialysis with severe chronic renal illness may have a decreased chance of developing nephrogenic systemic fibrosis if they start receiving hemodialysis as soon as a gadolinium-based contrast agent is given.
• Physical therapy: During physical therapy, stretching the afflicted limbs may assist in preserving the range of motion and stopping joint contractures from getting worse.
• Kidney transplant: Those who meet the eligibility conditions may potentially benefit from treatment for nephrogenic systemic fibrosis if their renal function improves after a kidney donation.
• Extracorporeal photopheresis using UVA light: This treatment requires that the blood be taken outside and treated with a drug that increases its sensitivity to UV radiation. Then the blood is put back into the body after being exposed to UV light. This treatment has made some people better.

These medications are still experimental, despite not being used yet. Negative consequences limit their use, even if some have shown benefit from them:

• Imatinib: Although this treatment has some potential to reduce skin thickness and tightness, more research is necessary.
• Pentoxifylline: This medication has a mixed track record of efficacy; it is said to increase circulation by decreasing blood thickness and stickiness (viscosity). Additional research is necessary.
• Sodium thiosulfate: Further research is necessary, although there might be an advantage to utilizing this medication.
• The higher dose of immune globulin is administered intravenously. Further research is necessary, although there might be an advantage to utilizing this medication.

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Prevention Of Nephrogenic Systemic Fibrosis

Avoiding older gadolinium-based contrast agents (group 1) can help prevent nephrogenic systemic fibrosis, but newer gadolinium-based contrast agents (group 2) are safer and do not provide an increased risk.

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