Nephrogenic systemic fibrosis

Nephrogenic systemic fibrosis
Classification and external resources
ICD-10	M35.5
ICD-9-CM	710.8
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Nephrogenic systemic fibrosis (NSF) or nephrogenic fibrosing dermopathy (NFD) is a rare and serious syndrome that involves fibrosis of skin, joints, eyes, and internal organs. The first cases were identified in 1997 and its cause is not fully understood. However, evidence suggests NSF is associated with exposure to gadolinium (with gadolinium-based MRI contrast agents being frequently used as contrast agents for magnetic resonance imaging (MRI)) in patients with severe kidney failure. Epidemiological studies suggest that the incidence of NSF is unrelated to gender, race, or age and it is not thought to have a genetic basis. A registry for NSF has identified about 335 cases as of 2011.

Most patients with NSF have undergone hemodialysis for kidney failure, some have never undergone dialysis and others have received only peritoneal dialysis. Many people with NSF have taken immunosuppressive medications and have other diseases, such as hepatitis C. Four of the seven gadolinium contrast agents approved by the U.S. Food and Drug Administration have been principally implicated in NSF, including gadodiamide, gadopentetate, and gadoversetamide. Gadobenate has also been associated with NSF, but further research has shown that gadobenate diglumine might be safe even in patients undergoing dialysis.

In NSF, patients develop large areas of hardened skin with fibrotic nodules and plaques. NSF may also cause joint contractures resulting in joint pain and limitation in range of motion. In its most severe form, NSF may cause severe systemic fibrosis affecting internal organs including the lungs, heart and liver.

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