Description

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

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

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

Symptoms

Nephrogenic systemic fibrosis can start from days to months, and even years after exposure to a former gadolinium-based contrast agent (group 1). Some of the signs and symptoms of nephrogenic systemic fibrosis may include:

  • Swelling and hardening of the skin
  • Redness or dark spots on the skin
  • The thickening and hardening of the skin, usually on the arms and legs, and sometimes on the body, but almost never on the face or in the head
  • The skin may feel "woody" and develop an orange-peel appearance
  • Burning, itching, or severe, sharp pains in the areas of participation of the
  • The thickening of the skin that inhibits the movement, resulting in the loss of the flexibility of the joints
  • Rarely, blisters, or ulcers.

In some persons, involvement of the muscles and organs of the body can cause:

  • Muscle weakness
  • Limitation of the mobility of the joints caused by muscle stiffness (contractures) in the arms, hands, legs and feet
  • Pain in the bones, especially in the hip bones or ribs
  • Reduced internal function of the organs, including the heart, the lungs, the diaphragm, gastrointestinal tract, or liver
  • Yellow plates on the white (sclera) of the eyes

The condition is usually long-term (chronic), but some people can improve. In some people, it can cause severe disability, even death.

Causes

The exact cause of nephrogenic systemic fibrosis is not fully understood. Fibrous connective tissue is formed in the skin and the connective tissue, leading to scarring of the tissues throughout the body, most commonly in the subcutaneous tissues and skin.

Exposure to higher gadolinium-based contrast agents (group 1) during magnetic resonance imaging (MRI) has been identified as a trigger for the development of this disease in people with kidney disease. This increased risk is thought to be related to the kidneys decreased ability to remove the contrast agent in the blood stream.

The Food and Drug Administration (FDA) recommended to avoid higher 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 the kidney disease and the exposure to higher gadolinium-based contrast agents (group 1), but the link is uncertain. These include:

  • The use of high-dose erythropoietin (EPO), a hormone that promotes the production of red blood cells, is often used to treat anemia
  • Recent vascular surgery
  • Blood clotting problems
  • Severe infection

Risk factors

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

  • You have moderate to severe kidney disease
  • Have had a kidney transplant, but they have compromised renal function
  • You are receiving haemodialysis or peritoneal dialysis
  • Have acute kidney injury

Prevention

The avoidance of higher gadolinium-based contrast agents (group 1) is the key to the prevention of nephrogenic systemic fibrosis, as the most recent of gadolinium-based contrast agents (group 2) they are more secure and are not associated with an increased risk.

Diagnosis

The diagnosis of nephrogenic systemic fibrosis is done with:

  • Physical examination for signs and symptoms of the disease, and the evaluation of a possible history of the mri using a gadolinium-based contrast agent with advanced kidney disease is present
  • A sample of tissue (biopsy) taken from the skin and the muscle
  • Other tests as needed that may indicate the involvement of the muscles and internal organs

Treatment

here there is no cure for nephrogenic systemic fibrosis, and the treatment is not consistent with success in arresting or reversing the progression of the disease. Nephrogenic systemic fibrosis occurs only rarely, making it difficult to conduct large studies.

Certain treatments have shown limited success in certain people with nephrogenic systemic fibrosis, but further research is needed to determine if these treatments help to:

  • Hemodialysis. In people with advanced chronic kidney disease who are on hemodialysis, hemodialysis immediately after the receipt of a gadolinium-based contrast agent can decrease the possibility of nephrogenic systemic fibrosis.
  • The physical therapy. Physical therapy helps to stretch the limbs may help slow the progression of contractures in the joints and preserving motion.
  • Kidney transplant. For the people who are suitable candidates, the improvement in renal function due to a kidney transplant can help to improve nephrogenic systemic fibrosis over time.
  • Extracorporeal photopheresis with ultraviolet A. This treatment consists in the removal of blood outside of the body and the blood treatment with a drug that sensitizes the ultraviolet light. The blood is exposed to ultraviolet light and returns it to the body. Some people have shown an improvement after receiving this therapy.

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

  • Imatinib (Gleevec). Although this treatment has shown some promise in the reduction of skin thickening and hardening, more research is needed.
  • Pentoxifylline (Pentoxil). It is not limited success with this medication, which theoretically decreases the thickness and viscosity (viscosity) of the blood, helping the circulation. More research is needed.
  • Sodium thiosulfate. Possible benefit has been shown that the use of this medicine, but more research is needed.
  • High-dose intravenous immunoglobulin. Possible benefit has been shown that the use of this medicine, but more research is needed.
Symptoms and treatment of Nephrogenic systemic fibrosis