Nephrogenic fibrosing dermopathy or nephrogenic systemic fibrosis? What we know and what we have to learn

Nephrogenic systemic fibrosis was first recognized as a unique entity in 1997 and subsequently defined in the literature in 2000 as a novel fibrosing disorder occurring in the setting of renal disease. Prevention, early recognition and treatment are essential to limiting its impact. The most important risk factors for developing NSF are chronic or significant acute kidney disease (especially dialysis dependent patients) and the administration of gadolinium containing contrast agents, agents that cause NFS by releasing free GD3 into tissues based on their pharmacokinetics. International commissions in drug control and medicinal products recommend to avoid gadolinium based contrast agents in patients with GFR < 30 mllminute/1,73 m(2). Unfortunately there is lack of universally effective therapy at this time and the literature is based on case reports and small case series. Recommendations to guide the use of gadolinium based contrast agents in patients with underlying kidney disease should be individualized and considered in consultation with the ordering physician, radiologist and nephrologist.