What is Nephrogenic Systemic Fibrosis: Causes, Symptoms, Treatment, Prevention, Diagnosis

Nephrogenic systemic fibrosis occurs in people suffering from advanced kidney failure. It is a rare disease that resembles skin diseases like scleroderma and scleromyxedema. It can affect the internal organs too like the heart, lungs, and kidneys. Nephrogenic systemic fibrosis affects the middle-aged people but has been confirmed in elderly and children too.

After getting exposure to gadolinium-containing contrast, nephrogenic systemic fibrosis can begin in days to months but progresses rapidly. The signs and symptoms of nephrogenic systemic fibrosis include:

• Tightening and swelling of the skin
• Skin thickening and hardening on the arms, legs and the body but not on the face.
• Woody feel of the skin and darkening of the skin
• Loss of joint flexibility
• Blisters and ulcers
• Muscle weakness
• Bone pain
• Reduced functioning of the internal organs like lungs, heart, liver, diaphragm
• Blood clots
• Yellow plaques on the sclera of the eyes.

The conditions are usually chronic though some people show improvement. In few cases of nephrogenic systemic fibrosis, it may result in death.

The exact cause of nephrogenic systemic fibrosis is not yet understood fully. The exposure of patients to the agents of gadolinium-containing contrast during MRI (Magnetic Resonance Imaging) triggers the development of nephrogenic systemic fibrosis. The FDA recommends avoiding exposure to those agents in people suffering from chronic kidney disease or injury. The other conditions that can promote nephrogenic systemic fibrosis include:

• Recent vascular surgery
• Blood clotting problems
• Use of high dose EPO (erythropoietin). EPO is a hormone which promotes the production of red blood cells, used for treating anemia.
• Severe infection.

Diagnosis of nephrogenic systemic fibrosis is made through the following methods:

• Physical examination of the signs and symptoms of nephrogenic systemic fibrosis, determination of a history of MRI using gadolinium in case of advanced kidney failure.
• Biopsy of the skin or muscle
• Other tests as may be required to diagnose any other differential diagnostic conditions.

There is no treatment for nephrogenic systemic fibrosis and no treatment is successful in a consistent way to halt or reverse the progress of nephrogenic systemic fibrosis. It occurs rarely so it is difficult to do large studies. Doctors have experienced the following treatments but more research is required to determine whether these treatments really help:

• Hemodialysis: Performing hemodialysis in people suffering from advanced kidney failure after receiving the gadolinium-containing contrast can reduce the possibility of nephrogenic systemic fibrosis.
• Physical Therapy for Nephrogenic Systemic Fibrosis: Physical therapy slows down the progress of joint contractures and retains movement.
• Ultraviolet A Phototherapy: Exposure of ultraviolet A to the skin reduces the thickening and hardening of the skin.
• Kidney Transplant to Treat Nephrogenic Systemic Fibrosis: Kidney transplant improves the renal function and may help to improve nephrogenic systemic fibrosis over a period of time.
• Extracorporeal Photopheresis for Nephrogenic Systemic Fibrosis: In this treatment, blood is drawn outside the body and is treated with a drug that can sensitize to ultraviolet light. After exposure of the blood to ultraviolet light, it is returned to the body. People suffering from nephrogenic systemic fibrosis have shown improvement with this therapy.
• Plasmapheresis to Treat Nephrogenic Systemic Fibrosis: In this procedure, the unwanted substances are removed from the blood, separating the plasma and solid blood cells, replacing the plasma with donor plasma, thereafter mixing with the original solid blood cells and sending it back to the body.

Medications like Pentoxil, Imatinib, and sodium thiosulfate have helped people too.

Prevention of nephrogenic systemic fibrosis is the best practice. Almost 40-50 percent of patients who undergo MRI receive gadolinium-contrast medium get this disease. FDA recommends that avoiding these agents can reduce the occurrence of nephrogenic systemic fibrosis.

Nephrogenic systemic fibrosis is a rare disease and all physicians who ask for MRI should be aware of using the gadolinium- contrast agents. The fatal effects of these agents need to be recognized too in the medical community. This disorder is in its infant stage and further research is required.