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nephrogenic systemic fibrosis
Nephrogenic systemic fibrosis (NSF) or nephrogenic fibrosing dermopathy 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.〔Thomsen HS. (2009) Nephrogenic systemic fibrosis: history and epidemiology'' Radiol Clin North Am.'' 47(5):827-31〕 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.〔(The NSF registry site ).〕
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.
==Systemic symptoms==
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.〔Kucher C, Steere, J, Elenitsas, R, Siegel, DL, Xu, X. (2006) Nephrogenic fibrosing dermopathy/nephrogenic systemic fibrosis with diaphragmatic involvement in a patient with respiratory failure. ''J Am Acad Dermatol'' 54: S31-34〕〔Krous H, Breisch, E, Chadwick, AE, Pinckney, L, Malicki, DM, Benador, N. (2007) Nephrogenic systemic fibrosis with multiorgan involvement in a teenage male after lymphoma, Ewing's sarcoma, end-stage renal disease, and hemodialysis. ''Pediatr Dev Pathol'' 10: 395-402.〕
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