Nephrogenic Systemic Fibrosis

National Organization for Rare Disorders, Inc.

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It is possible that the main title of the report Nephrogenic Systemic Fibrosis is not the name you expected.

Disorder Subdivisions

  • None

General Discussion

Nephrogenic systemic fibrosis (NSF) is a rare disorder that occurs in some individuals with reduced kidney function, who have been exposed to an intravenous contrast material that contains gadolinium. A contrast material is a dye that is sometimes used during magnetic resonance imaging (MRI). The term, fibrosis, refers to the thickening and scarring of connective tissue, most often the consequence of inflammation or injury. NSF is characterized by thickening and hardening (fibrosis) of the skin, subcutaneous tissues, and, sometimes, underlying skeletal muscle. The arms and legs are most often affected. In some cases, the skin on the trunk can also become involved. This proliferation of fibrotic tissue may become systemic, extending to other areas including the smooth, delicate membrane that surrounds the lungs (pleura), the sac surrounding the heart (pericardium), the thin sheet of muscle that aids respiration by moving up and down when breathing (diaphragm), and the outermost layer (dura mater) of the three membranes covering the brain and spinal cord.

NSF was thought to predominantly involve the skin and was originally referred to as nephrogenic fibrosing dermopathy. However, it is now known that it may involve several internal organs (systemic disorder), potentially leading to a progressive and severe disease course. NSF has not been reported in individuals with normal kidney function.

Since September of 2010, the U.S. Food and Drug Administration (FDA) has required that gadolinium-based contrast agents (GBCA) carry a warning on their labels about the risk of NSF when administered in certain individuals with kidney disease. In addition, the FDA also stated that three specific gadolinium-based contrast agents (Magnevist ®, OmniscanTM, and Optimark ®) were contraindicated in individuals with severe chronic kidney disease or acute kidney injury. These three agents were the ones most often associated with NSF.

Supporting Organizations

Global Fibrosis Foundation

Yale Dermatopathology Service
15 York Street, LMP 5031
New Haven, CT 06520-8059
Tel: (727)433-8180

National Kidney Foundation

30 East 33rd Street
New York, NY 10016
Tel: (212)889-2210
Fax: (212)689-9261
Tel: (800)622-9010

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Last Updated:  4/2/2014
Copyright  2014 National Organization for Rare Disorders, Inc.