Nephrogenic systemic fibrosis in advanced chronic kidney disease: a single hospital's experience in Taiwan

Nephrogenic systemic fibrosis (NSF) or nephrogenic fibrosing dermopathy (NFD) clinically resembles scleromyxedema which develops in the setting of advanced chronic kidney diseases. Limited data exist about its epidemiology in Asian countries. A total of 153 magnetic resonance imaging (MRI) examinations, including 81 contrast-enhancement, were identified in 127 patients with advanced chronic kidney disease at stage five undergoing MRI or angiography examination between January 2005 and July 2007, in our hospital. The diagnosis of NFD/NSF was established based on clinical manifestation and histopathology. NFD/NSF was diagnosed in none of the 105 patients on haemodialysis but in one of the 22 patients on peritoneal dialysis. This 24-year-old woman was a case of systemic lupus erythematosus since age 15 and who developed skin lesions two months before the initiation of peritoneal dialysis but nine months after four exposures to gadodiamide during MRI study. The skin condition had significantly improved within three months under a combination regimen of systemic pentoxifylline and topical clobetasol propionate ointment, with further amelioration during subsequent treatment with colchicine. Our results lend support to the predisposition of gadolinium-containing contrast agents to the development of NFD/NSF in patients with advanced renal failure, even before the initiation of dialysis. The cause of a lower incidence rate in our series remains to be determined.