[Nephrogenic systemic fibrosis--new clinical entity]

Abstract

Nephrogenic systemic fibrosis (NSF) is a new clinical entity, which was first described in 2000. It is associated with fibrosis of the skin and visceral organs in patients with impaired renal function. There are over 260 reported cases of NSF with hundreds still not reported. The application of gadolinium-containing contrast media in patients with decreased renal function can lead to gadolinium deposition in various tissues, which induces the fibrotic process. In our previous study, that involved over 20,000 patients with postcontrast examination, we did not have any case of NSF. The primary manifestation of NSF is the skin findings. The skin is raised and thickened with edema. The most frequent areas of involvement are the lower extremity, followed by the upper extremity and trunk. Disabling and painful contractures are prominent in NSF. Chronically, the skin changes may appear as nodules, patches, hyperpigementation, and indurations. Fibrosis can involve joints, skeletal muscles, bone, and almost any tissue in the body. Risk factors include the severity of renal impairment, major surgery, metabolic acidosis, and other proinflammatory conditions. The data in many investigations strongly suggest that stability of the gadolinium-complex is a key factor for the development of NSF. Gadodiamid (Omniscan) is non-ionic linear structure Gd-complex with the highest risk of releasing Gd-ions in vivo, while the risk is significantly reduced with ionic linear agents, such as Magnevist and Multhance, and even further reduced with macrocyclic agents, such Dotarem and Gadovist. Currently there is no effective treatment, so prevention is the only way to avoid this serious illness.