Nephrogenic systemic fibrosis is a rare connective tissue disorder affecting people with kidney disease, caused by exposure to gadolinium used in diagnostic imaging scans. Symptoms include hard, discolored skin, joint pain, and muscle weakness. There is no cure, but treatment can relieve symptoms. Diagnosis is made through physical evaluation and skin biopsy.
Nephrogenic systemic fibrosis is a very rare connective tissue disorder that affects some people with acute or chronic kidney disease. The exact causes are not well understood, but there appears to be a causal link between exposure to the element gadolinium and the onset of symptoms. People who have the disorder typically develop patches of hard, tight, and discolored skin as well as joint pain and muscle weakness after dialysis treatments for their kidney problems. Nephrogenic systemic fibrosis can be debilitating as the skin and joints become very stiff. There are no reliable cures for the condition, but ultraviolet light therapy and topical creams can help relieve symptoms in some patients.
Gadolinium is commonly used as a traceable dye for diagnostic imaging scans. Gadolinium-based fluorescent contrast agents (GBCAs) are used in many different types of MRI scans, including angiography, to help radiologists monitor blood flow through vessels and organs. Doctors aren’t exactly sure how GBCAs affect nephrogenic systemic fibrosis, but the correlation is very well documented. The most likely explanation is that gadolinium stimulates the activity of latent, immature connective tissue cells called fibrocytes. When fibrocytes become active, they enter the bloodstream and trigger adverse responses in connective tissue throughout the body.
A person who has nephrogenic systemic fibrosis can experience a variety of symptoms. Patches of skin on the abdomen, chest, back, or extremities may gradually harden and thicken over time. The patches tend to turn dark red and cause constant itching and burning sensations. When deeper connective tissue is affected, major joints can become stiff, swollen, and very painful. Some people have trouble bending and straightening their hips, knees, elbows, and fingers.
Muscle weakness combined with joint problems can also make walking impossible. Many people are confined to beds or wheelchairs as their symptoms get progressively worse. Life-threatening complications can arise if fibrosis spreads to the kidneys, liver, heart, or other major organ systems in the body.
A specialist can diagnose nephrogenic systemic fibrosis by evaluating physical symptoms and reviewing the patient’s history of dialysis procedures and imaging tests. A skin biopsy can be taken from an affected patch of skin to confirm the diagnosis. Since the condition cannot be reversed, therapeutic efforts are primarily aimed at relieving symptoms. There is some clinical evidence to support the benefits of light therapy in softening the skin and healing difficult areas, but the true effectiveness of such procedures has not been verified. Most patients are given topical creams to relieve itching and continue to receive treatment for their kidney ailments in the hope that the symptoms will eventually resolve.
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