Nephrogenic systemic fibrosis

Overview

Nephrogenic systemic fibrosis is a rare and debilitating disease that affects people with advanced kidney failure, often with or without dialysis. The condition is characterized by the progressive thickening and darkening of large areas of the skin, which can be disfiguring and disabling. Nephrogenic systemic fibrosis can also affect internal organs, such as the heart and lungs, and can cause a disabling shortening of muscles and tendons in the joints (joint contracture).

Types of Nephrogenic systemic fibrosis

There is only one type of nephrogenic systemic fibrosis, which is characterized by the progressive thickening and darkening of the skin.

Causes of Nephrogenic systemic fibrosis

Nephrogenic systemic fibrosis is caused by exposure to gadolinium-based contrast agents, which are used in magnetic resonance imaging (MRI) scans. The condition is often seen in people with advanced kidney failure who have undergone multiple MRI scans.

Risk Factors of Nephrogenic systemic fibrosis

The risk factors for developing nephrogenic systemic fibrosis include:

1. Advanced kidney failure: People with advanced kidney failure are at a higher risk of developing nephrogenic systemic fibrosis.
2. Dialysis: People who receive dialysis are at a higher risk of developing nephrogenic systemic fibrosis.
3. Gadolinium-based contrast agents: Exposure to gadolinium-based contrast agents increases the risk of developing nephrogenic systemic fibrosis.
4. Age: The risk of developing nephrogenic systemic fibrosis increases with age.

Symptoms of Nephrogenic systemic fibrosis

Preventions of Nephrogenic systemic fibrosis

Preventing nephrogenic systemic fibrosis involves avoiding exposure to gadolinium-based contrast agents and taking steps to reduce the risk of developing advanced kidney failure.

1. Avoiding gadolinium-based contrast agents: Avoiding exposure to gadolinium-based contrast agents is essential for preventing nephrogenic systemic fibrosis.
2. Managing kidney failure: Managing kidney failure through proper treatment and care can help reduce the risk of developing nephrogenic systemic fibrosis.

Diagnosis of Nephrogenic systemic fibrosis

Diagnosing nephrogenic systemic fibrosis typically involves a comprehensive evaluation that includes:

1. Physical examination: A physical examination is performed to assess the extent of skin thickening and darkening.
2. Imaging tests: Imaging tests, such as MRI scans, may be performed to assess the extent of joint contracture.
3. Laboratory tests: Laboratory tests may be performed to assess kidney function and other organ functions.

Treatment for Nephrogenic systemic fibrosis

Treating nephrogenic systemic fibrosis involves managing the symptoms and reducing the progression of the disease.

1. Supportive therapy: Supportive therapy, such as physical therapy, occupational therapy, and pain management, may be necessary to manage symptoms.
2. Medications: Medications may be prescribed to reduce inflammation and prevent further progression of the disease.
3. Surgery: In severe cases, surgery may be necessary to correct joint contracture or other deformities.

When to seek medical attention ?

If you are experiencing symptoms of nephrogenic systemic fibrosis or have been exposed to gadolinium-based contrast agents, it is essential to see a doctor for evaluation and treatment.