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

2009

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…