Gadolinium-based MR contrast agents and nephrogenic systemic fibrosis.

摘要

Nephrogenic systemic fibrosis (NSF), described in 2000 (1), is an emerging systemic disorder characterized by widespread tissue fibrosis. Originally known as nephrogenic fibrosing der-mopathy because of its dominant cutaneous findings, the nomenclature was revised in recent years to reflect an increased understanding of its systemic effects (1,2). While the precise cause of NSF remains a mystery, it is known to occur only in patients with renal disease-generally in those requiring dialysis. NSF may develop rapidly and can sometimes result in patients becoming confined to a wheelchair within a few weeks. More commonly, the skin thickening is insidious and can be confused clinically with peripheral edema. Pathophysiologically, NSF results in increased tissue deposition of collagen, commonly resulting in thickening and hardening of the skin of the extremities and often culminating in immobility and contractures of the joints. In some patients, there is clinical involvement of other tissues (lung, skeletalmuscle, heart, diaphragm, esophagus, etc), although the patient may not be clinically symptomatic (3). While NSF sometimes stabilizes, it rarely spontaneously remits. No consistently effective therapy exists, although rapid correction in renal function (by medical or surgical means) generally results in a cessation of progression and often in a reversal of symptoms (4).