Abstract

Nephrogenic systemic fibrosis (NSF) which used to be named nephrogenic fibrosing dermopathy is a new disease appears after 1997. It is characterized by scleroderma-like skin fibrosis and may systemically involve other organs, including heart, lung, liver, and striated muscle. The disease might be triggered by the exposure to Gadolinium (Gd)-based magnetic resonance image contrast agents, and develops exclusively in patients with various stage of renal failure. Gadodiamide (Omniscan(supercrispt ®)) is among them, most frequently reported in the cases of NSF. The gold standard of diagnosis is deep skin biopsy which showed CD 34/procollagen dual positive in spindle cells, and absence of inflammation. There are several differential diagnoses to be made, such as cellulitis, drug reaction, scleroderma, scleromyxedema, and systemic sclerosis. To date, the disease mechanism is still unclear and there is no proven treatment for NSF. Therefore prevention of NSF should be emphasized. Food and Drug Administration recommends avoidance of Gd-containing contrast agents in patients with acute and chronic renal failure (glomerular filtration rate<30ml/min/1.73m^2), unless the diagnostic information is essential and cannot be replaced by other image studies. The dose of Gd should not exceed the recommendation and there must be sufficient time for Gd elimination before readministration. Prompt hemodialysis (HD) should be considered in patients receiving dialysis after Gd exposure as Gd can be effectively eliminated via HD.

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