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Journal of the American Academy of Dermatology
Volume 61, n° 5
pages 868-874 (novembre 2009)
Doi : 10.1016/j.jaad.2009.03.040
Case & Reviews

Nephrogenic systemic fibrosis–a rapidly progressive disabling disease with limited therapeutic options
 

Gisela Schieren, MD a, , Nikolaus Wirtz, MD b, Peter Altmeyer, MD c, Lars Christian Rump, MD a, Stefan Markus Weiner, MD b, , Alexander Kreuter, MD c,
a Department of Nephrology, University Hospital, Heinrich-Heine University Düsseldorf, Düsseldorf, Germany 
b Department of Rheumatology, Immunology, and Nephrology, Krankenhaus der Barmherzigen Brüder Trier, Teaching Hospital of the University of Mainz, Trier, Germany 
c Department of Dermatology, Josefs-Hospital, Ruhr University Bochum, Bochum, Germany 

Reprint requests: Gisela Schieren, MD, Department of Nephrology, University Hospital, Heinrich-Heine University Düsseldorf, Moorenstrasse 5, 40225 Düsseldorf, Germany.
Abstract
Background

Nephrogenic systemic fibrosis (NSF) affects some patients on dialysis after gadolinium contrast agent–enhanced magnetic resonance imaging. It is characterized by progressive skin fibrosis of the extremities, sometimes including the trunk and internal organs.

Methods

The clinical course of 10 patients with biopsy-proven NSF was analyzed retrospectively with regard to gadolinium exposition, disease onset, and progression of NSF with special emphasis on physical mobility and impact of different therapeutic approaches.

Results

Despite physiotherapy and different additional therapeutic approaches (eg, immunosuppression, ultraviolet A-1 phototherapy, or extracorporal photopheresis) all patients developed progressive skin fibrosis of the lower extremities, sometimes including the trunk and arms. Kidney transplantation led to a slow improvement of skin lesions in one patient. Nine patients developed progressive joint contractures, and 8 patients became wheelchair bound within 12 months after disease onset and became dependent on the support of family members or a nursing service.

Limitations

Retrospective analysis in a relatively small number of patients is a limitation.

Conclusion

NSF appears to be a rapidly progressive disabling disease with limited therapeutic options.

The full text of this article is available in PDF format.

Key words : disabling disease, end-stage renal disease, gadolinium, joint contraction, nephrogenic systemic fibrosis

Abbreviations used : DTPA, Gd, MRI, NSF



 Funding sources: None
 Conflicts of interest: None declared.

  Split senior authorship, as both authors contributed equally to the article.


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