Overview
Nephrogenic systemic fibrosis is a rare disease that primarily affects individuals with advanced kidney failure, including those on dialysis. The disease causes thickening and darkening of the skin, as well as internal organ involvement such as the heart and lungs. It can also result in joint contractures due to muscle and tendon shortening. The exact cause of nephrogenic systemic fibrosis is not fully understood, but it is thought to involve the production of excess fibrous connective tissue. Exposure to older gadolinium-based contrast agents (group 1) during magnetic resonance imaging (MRI) has been identified as a trigger for developing this condition in people with kidney disease. This has led to a significant reduction in cases by avoiding these contrast agents. Newer gadolinium-based contrast agents (group 2) are not associated with an increased risk.
Understanding the disease
Symptoms
The symptoms of nephrogenic systemic fibrosis may include swelling and tightening of the skin, reddened or darkened patches on the skin, blood in the urine, foamy urine, pain on one or both sides of the back below the ribs, swelling in the hands and feet (edema), high blood pressure, weakness, and tiredness. If the disease leads to kidney failure, additional symptoms may appear, such as rashes, muscle cramps, nausea, loss of appetite, metallic taste in the mouth, and confusion.
Development
Nephrogenic systemic fibrosis (NSF) develops due to the formation of fibrous connective tissue in the skin and connective tissues. This leads to scarring of tissue throughout the body, primarily in the skin and subcutaneous tissues. The exact cause of NSF is not fully understood, but exposure to older gadolinium-based contrast agents (group 1) during magnetic resonance imaging (MRI) has been identified as a trigger in people with kidney disease. The reduced ability of the kidneys to remove the contrast agent from the bloodstream is thought to be responsible for the increased risk. Newer gadolinium-based contrast agents (group 2) are not associated with an increased risk of systemic nephrogenic fibrosis. Recognition of this link has significantly reduced the incidence of NSF.
Assessment and Diagnosis
Triage
If you suspect nephrogenic systemic fibrosis, it is important to consult a doctor as soon as possible, especially if you have advanced kidney disease or have been exposed to older gadolinium-based contrast agents during an MRI or other imaging studies. Early diagnosis and treatment can help manage the symptoms and potential complications of the disease.
Diagnosis
The diagnosis of nephrogenic systemic fibrosis involves a physical exam to check for signs and symptoms of the disease, along with an evaluation of the individual's history of MRI using a gadolinium-based contrast agent when advanced kidney disease is present. A sample of tissue (biopsy) taken from the skin and muscle is also taken for examination. Other tests may be conducted as needed to indicate involvement of muscles and internal organs.
Management and Treatment
Complications
Potential complications of nephrogenic systemic fibrosis include disabling shortening of muscles and tendons in the joints (joint contracture), internal organ involvement such as affecting the heart and lungs, and severe disability, which in some cases can lead to death.
Prevention
Prevention of nephrogenic systemic fibrosis primarily involves avoiding exposure to older gadolinium-based contrast agents (group 1) during magnetic resonance imaging (MRI) and other imaging studies. The Food and Drug Administration (FDA) recommends avoiding these contrast agents in people with acute kidney injury or chronic kidney disease. If you have kidney disease, it is important to inform your healthcare provider about your condition before undergoing any imaging procedures that may require contrast agents. Additionally, managing your kidney disease through proper treatment and medication can help reduce the risk of developing nephrogenic systemic fibrosis. Maintaining a healthy lifestyle, including regular exercise and a balanced diet, can also contribute to overall kidney health and potentially lower the risk of the disease. It is essential to consult with your healthcare provider about your individual risk factors and the best strategies for prevention.
Home Remedies
It is important to consult a healthcare professional for proper diagnosis, treatment, and management of nephrogenic systemic fibrosis.