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    Nephrogenic Systemic Fibrosis

    Important
    It is possible that the main title of the report Nephrogenic Systemic Fibrosis is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

    Synonyms

    • NSF
    • gadolinium-associated nephrogenic systemic fibrosis
    • nephrogenic fibrosing dermopathy

    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.

    Resources

    National Kidney Foundation
    30 East 33rd Street
    New York, NY 10016
    Tel: (212)889-2210
    Fax: (212)689-9261
    Tel: (800)622-9010
    Email: info@kidney.org
    Internet: http://www.kidney.org

    Global Fibrosis Foundation
    250 Main Street
    Suite 425775
    Cambridge, MA 02142
    Tel: (727)433-8180
    Tel: (800)872-6874
    Email: globalfibrosis@gmail.com
    Internet: http://www.globalfibrosis.com

    For a Complete Report:

    This is an abstract of a report from the National Organization for Rare Disorders (NORD). A copy of the complete report can be downloaded free from the NORD website for registered users. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational therapies (if available), and references from medical literature. For a full-text version of this topic, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".

    The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

    It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report

    This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

    For additional information and assistance about rare disorders, please contact the National Organization for Rare Disorders at P.O. Box 1968, Danbury, CT 06813-1968; phone (203) 744-0100; web site www.rarediseases.org or email orphan@rarediseases.org

    Last Updated: 4/2/2014
    Copyright 2011, 2014 National Organization for Rare Disorders, Inc.

    WebMD Medical Reference from the National Organization for Rare Disorders

    Last Updated: May 28, 2015
    This information is not intended to replace the advice of a doctor. Healthwise disclaims any liability for the decisions you make based on this information.

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