Format

Send to

Choose Destination
See comment in PubMed Commons below
J Am Acad Dermatol. 2009 Nov;61(5):868-74. doi: 10.1016/j.jaad.2009.03.040.

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

Author information

  • 1Department of Nephrology, University Hospital, Heinrich-Heine University Düsseldorf, Düsseldorf, Germany. gisela.schieren@med.uni-duesseldorf.de

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.

PMID:
19836645
DOI:
10.1016/j.jaad.2009.03.040
[PubMed - indexed for MEDLINE]
PubMed Commons home

PubMed Commons

0 comments
How to join PubMed Commons

    Supplemental Content

    Full text links

    Icon for Elsevier Science
    Loading ...
    Support Center