Nephrogenic systemic fibrosis: a gadolinium-associated fibrosing disorder in patients with renal dysfunction.

摘要

Nephrogenic systemic fibrosis (NSF) is a debilitating fibrosing disorder that develops in patients with underlying kidney disease following exposure to gadolinium-containing contrast agents. NSF presents with cutaneous hyperpigmentation and induration and joint contractures, but fibrosis may also develop in other organs. NSF has been observed in up to 18% of patients receiving chronic haemodialysis and also may occur in individuals with stages 3 and 4 chronic kidney disease and, occasionally, in individuals who had experienced acute renal failure. Mortality is increased significantly among individuals with NSF. Although no medical treatment has been proved to be universally effective in patients with NSF, imatinib mesylate shows potential as a therapeutic agent and is currently being studied in these patients.