Guide To Treating Nephrogenic Systemic Fibrosis

April 17, 2023

Nephrogenic systemic fibrosis is a progressive disease that affects the skin and vital organs. Nearly all cases of this condition develop in patients who have severe chronic renal insufficiency. For many of these patients, exposure to gadolinium (a contrast agent sometimes injected for MRI scans) triggers the disease, and symptoms can develop within days or months of exposure. Unfortunately, nephrogenic systemic fibrosis advances rapidly, and it can be fatal. Initial symptoms typically include swelling and hardening of the skin on the arms and legs. Patients frequently experience a thickening of the skin that impedes movement, and the areas involved may feel itchy and painful. If nephrogenic systemic fibrosis involves muscles and organs, symptoms may include blood clots, muscle weakness, bone pain, and yellowing of the whites of the eyes (the sclera). The disease can impair the function of the heart, lungs, diaphragm, liver, and gastrointestinal tract. To diagnose this condition, doctors perform a physical exam, and both skin and muscle biopsies are taken.

The treatment methods described below are typically recommended for nephrogenic systemic fibrosis.

Kidney Transplant

A kidney transplant may be appropriate for certain patients with nephrogenic systemic fibrosis. The transplant improves overall kidney function, and this can improve nephrogenic systemic fibrosis symptoms. Kidney transplants may provide a higher quality of life, lower treatment cost, lower risk of dying, and fewer dietary restrictions as compared to dialysis. Kidney transplants can be performed using a live donor, and a single donated kidney replaces two failing kidneys. Risks associated with this procedure include failure or rejection of the transplanted kidney, blood clots, infection, and a leak or blockage in the ureter. After having this procedure, patients typically stay in the hospital for up to one week, and they are closely monitored with blood tests several times a week for the first few weeks after returning home. Patients will be required to take anti-rejection medication for the rest of their lives. These medicines can cause bone thinning, puffiness, weight gain, hypertension, and high cholesterol.

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Plasmapheresis

Similar to dialysis, plasmapheresis is a process that filters the blood, removing specific antibodies from a portion of the blood known as plasma. The procedure makes it possible for a patient to safely receive a kidney transplant from a donor who has an incompatible blood type. To begin the procedure, a nurse places a needle and intravenous (IV) line in each of the patient's arms. Alternatively, a central line or port access can be used instead. Next, an anticoagulant is added to the patient's blood to prevent the formation of clots, and both the blood and the anticoagulant flow into a machine. Inside the machine, the blood is separated into plasma and other components. Next, replacement fluid is added to replace the plasma that has been separated. Finally, the replacement fluid and blood components (minus the plasma) are returned to the patient's body through the second IV line. While receiving plasmapheresis, patients may experience low blood pressure, fatigue, dizziness, and a tingling sensation in the fingers or mouth. Patients will be monitored closely during the procedure, and they should notify a nurse immediately if they develop any of these side effects.

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Hemodialysis

Hemodialysis is one of the most common forms of dialysis, and it is often performed at specialist dialysis centers. Some patients can receive hemodialysis at home. Patients who have been exposed to gadolinium may be able to reduce their risk of developing nephrogenic systemic fibrosis if they have hemodialysis immediately following their MRI scan. Normally, this is only recommended for patients who already have severely reduced kidney function and who are already receiving dialysis.

Hemodialysis filters the blood through a machine called a dialyzer. First, two needles are placed in one arm, and patients can place their own needles if they've been trained by staff. The needles are connected to lines that flow into the dialyzer. Inside the machine, waste products are removed from the blood, and the filtered blood returns to the patient via the second line. Depending on the patient's particular needs and dialysis schedule, they may have hemodialysis sessions three, five, or seven days a week.

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Selected Medications

Nephrogenic systemic fibrosis patients may also be treated with selected medications that can ease symptoms and improve quality of life. For example, a medication called imatinib has shown some success in reducing the skin thickening and tightness that commonly occur with this disease. Another medication, pentoxifylline, may promote better circulation for patients by reducing blood viscosity (thickness). Sodium thiosulfate may be beneficial for some patients.

These medications are still under investigation, and patients using them should be aware of potential side effects. Imatinib can cause fever, rashes, nausea, vomiting, swelling, bleeding problems, bone pain, and muscle cramps. Patients taking pentoxifylline may develop flushing, blurry vision, bloating, upset stomach, and diarrhea. Potential side effects of sodium thiosulfate include tinnitus, joint pain, hallucinations, mental changes, and agitation. Patients and their caregivers should report any side effects to the prescribing physician as soon as possible.

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Ultraviolet A Phototherapy

Ultraviolet A phototherapy involves the use of UV-A rays, and the treatment can reduce the redness and inflammation associated with the skin thickening nephrogenic systemic fibrosis often causes. Patients may need to have treatments several times a week for at least two months to effectively manage their symptoms. After applying a moisturizer or oil to the affected areas and putting on protective eyewear, the patient enters a cabinet surrounded by lights on all sides. The treatment provider turns the lights on for a few minutes, and the lights may be turned on for increasing lengths of time at each subsequent treatment. Nephrogenic systemic fibrosis patients will have their skin closely monitored while receiving phototherapy, and they should remain vigilant for possible side effects, including sunburn.

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