Abstract
Nephrogenic systemic fibrosis (NSF) - previously termed nephrogenic fibrosis dermopathy - is a newly recognized disorder occurring only in patients with renal failure. Exposure to gadolinium-containing contrast agents used for magnetic resonance imaging has been associated with subsequent development of NSF. This disease is characterised by swelling and tightening of the skin, mostly at the limbs. In addition, internal organs may be involved, which may ultimately cause death in rare cases. Skin biopsy showing fibrous tissue and spindle cells positive for CD34, and factor XIIIa as well as CD68-positive macrophages confirms the diagnosis. The main therapeutic goal is restoration of renal function by renal transplantation or recovery from acute renal failure, whenever possible. There are few data regarding other measures. The best available evidence for some therapeutic effect relates to physiotherapy and extracorporal photopheresis. Gadodiamide and gadopentetate-dimeglumin must not be used in stage 4 and 5 renal failure. Other gadolinium-containing contrast media must be used with extreme caution in patients with advanced renal failure.
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