Nephrogenic systemic fibrosis. A debilitating disease causing fibrosis of the skin and inner organs in patients with kidney failure.
Nephrogenic systemic fibrosis (NSF) is a rare and debilitating disease which affects patients with kidney failure. The most obvious manifestation is fibrosis of the skin, but it also frequently involves the locomotor system and the inner organs. An association has been found with the administration of gadolinium-containing contrast agents, which are given to provide enhanced contrast during magnetic resonance imaging. It is thought that unstable chelate complexes release toxic gadolinium. Other triggers or co-triggers may also be relevant. No effective treatment currently exists for NSF, so prevention of the disease is of the utmost importance. If gadolinium-containing contrast agents need to be administered to patients who have kidney failure, a cyclic agent should be used, and the dosage should be as low as possible. Although no proof is yet available that hemodialysis prevents NSF, it is effective in the clearance of gadolinium and should therefore be considered as a treatment immediately after the imaging.
Pieringer
H
H
Section of Rheumatology and Nephrology, 2nd Department of Medicine, General Hospital Linz, Austria. herwigpi@yahoo.com
Biesenbach
G
G
D006801:Humans; D007596:Joints / Q000473:pathology*; D008279:Magnetic Resonance Imaging; D054989:Nephrogenic Fibrosing Dermopathy / Q000473:pathology* / Q000503:physiopathology* / Q000628:therapy; D051437:Renal Insufficiency / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D012867:Skin / Q000473:pathology*