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

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.

• Authors: Gisela Schieren, Nikolaus Wirtz, Peter Altmeyer, Lars Christian Rump, Stefan Markus Weiner, Alexander Kreuter
• Journal: Journal of the American Academy of Dermatology (J Am Acad Dermatol) Vol. 61 Issue 5 Pg. 868-74 (Nov 2009) ISSN: 1097-6787 [Electronic] United States
• PMID: 19836645 (Publication Type: Case Reports, Journal Article, Review)

Chemical References

• Gadolinium

Topics

• Adult
• Biopsy
• Disease Progression
• Female
• Gadolinium (adverse effects)
• Humans
• Hydronephrosis (complications, pathology, therapy)
• Magnetic Resonance Imaging
• Nephrectomy
• Nephrogenic Fibrosing Dermopathy (chemically induced, pathology, therapy)
• Renal Dialysis