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STATE-OF-THE-ART PAPER

Nephrogenic Systemic Fibrosis

Pathogenesis, Diagnosis, and Therapy

Andreas Kribben, MD^_*,_*, Oliver Witzke, MD^_*, Uwe Hillen, MD, Jörg Barkhausen, MD, Anton E. Daul, MD^|| and Raimund Erbel, MD

^_* Department of Nephrology, University Duisburg-Essen, Essen, Germany
Department of Dermatology, University Duisburg-Essen, Essen, Germany
Institute for Diagnostic and Interventional Radiology and Neuroradiology, University Duisburg-Essen, Essen, Germany
Department of Cardiology, University Duisburg-Essen, Essen, Germany
^|| Department of Internal Medicine, Division of Nephrology, Elisabeth Hospital Essen, Essen, Germany

Manuscript received August 11, 2008; revised manuscript received December 3, 2008, accepted December 8, 2008.

^_* Reprint requests and correspondence: Dr. Andreas Kribben, Department of Nephrology, University Duisburg-Essen, Hufelandstrasse 55, 45122 Essen, Germany (Email: andreas.kribben{at}uni-due.de).

Nephrogenic systemic fibrosis (NSF) is a newly recognized disorder occurring exclusively in patients with renal failure. Exposure to gadolinium-based magnetic resonance (MR) contrast media has been associated with subsequent development of NSF. Nephrogenic systemic fibrosis is characterized by skin induration preferentially affecting the extremities. In addition, involvement of internal organs occurs, which leads ultimately to death. Skin biopsy is important for confirmation of the diagnosis. The main therapeutic goal is restoration of renal function. To reduce the risk of NSF, renal function must be determined before exposure to gadolinium-containing MR contrast agents. Gadolinium-based MR contrast media should be avoided in the presence of advanced renal failure with estimated glomerular filtration rate below 30 ml/min/1.73 m², unless the diagnostic information is essential and not available with noncontrast magnetic resonance imaging techniques. The recommended dose of contrast agent should not be exceeded. In addition, a sufficient period of time for elimination of the contrast agent from the body should be allowed before readministration of the contrast agent.

Key Words: gadolinium • magnetic resonance imaging • nephrogenic systemic fibrosis • renal failure

Abbreviations and Acronyms   AKI = acute kidney injury   CKD = chronic kidney disease   FDA = Food and Drug Administration   GFR = glomerular filtration rate   MRA = magnetic resonance angiography   MRI = magnetic resonance imaging   NSF = nephrogenic systemic fibrosis

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