title: 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.
  
  
  CONCLUSIONS
  Retrospective analysis in a relatively small number of patients is a limitation.
  
  
  CONCLUSIONS
  NSF appears to be a rapidly progressive disabling disease with limited therapeutic options.
authors.affiliation: Department of Nephrology, University Hospital, Heinrich-Heine University Düsseldorf, Düsseldorf, Germany. gisela.schieren@med.uni-duesseldorf.de
authors.forename: Gisela
authors.initials: G
authors.lastname: Schieren
authors.forename: Nikolaus
authors.initials: N
authors.lastname: Wirtz
authors.forename: Peter
authors.initials: P
authors.lastname: Altmeyer
authors.forename: Lars Christian
authors.initials: LC
authors.lastname: Rump
authors.forename: Stefan Markus
authors.initials: SM
authors.lastname: Weiner
authors.forename: Alexander
authors.initials: A
authors.lastname: Kreuter
mesh_terms: D000328:Adult; D001706:Biopsy; D018450:Disease Progression; D005260:Female; D005682:Gadolinium / Q000009:adverse effects; D006801:Humans; D006869:Hydronephrosis / Q000150:complications* / Q000473:pathology / Q000628:therapy; D008279:Magnetic Resonance Imaging; D009392:Nephrectomy; D054989:Nephrogenic Fibrosing Dermopathy / Q000139:chemically induced / Q000473:pathology* / Q000628:therapy*; D006435:Renal Dialysis
chemical_list: D005682:Gadolinium