Nephrogenic systemic fibrosisephrogenic fibrosing dermopathy: a primer for radiologists.

摘要

Nephrogenic systemic fibrosis (NSF) is a rare, idiopathic systemic fibrosing disorder that is predominantly characterized by a clinicopathologically distinct symmetric dermopathy and debilitating joint contractures. The condition affects patients with renal insufficiency and appears to show improvement with prompt restoration of renal function. Although NSF was initially reported in the United States as nephrogenic fibrosing dermopathy, it has subsequently been described in patients in Europe and Asia. More than 215 cases of NSF have been reported in the nephrogenic fibrosing dermopathy/NSF registry that is currently maintained at the Yale University. Recent reports suggest a possible etiologic link between systemic administration of gadolinium-containing magnetic resonance imaging contrast agents in patients with renal insufficiency and the development of NSF.