WHAT IS NSF?

WHAT IS NSF?
Nephrogenic systemic fibrosis, also known as nephrogenic fibrosing dermopathy, is a rare disease first recognized in 1997 and first characterized in 2000. Until May 2007, only about 215 cases had been reported to the International Center for Nephrogenic Fibrosing Dermopathy Research (ICNFDR) and the FDA [2, 3]
Risk Factors
To date, NSF has only been reported in patients with renal insufficiency. There is no link between the duration of renal disease and development of NSF.
A strong link has been found between administration of gadolinium-based contrast and development of NSF. The FDA reported in May 2007 that of the 215 cases of NSF reported to date, detailed histories were reviewed for 75, all of whom received gadolinium prior to development of the disease. The form of gadolinium most often associated with NSF is gadodiamide, although the “FDA believes that there is a potential for NSF/NFD to occur with the use of any of the approved gadolinium-based contrast agents” [2].
NSF often develops in patients who have recently had vascular thrombosis or vascular surgery.
There may be an association with other inflammatory conditions (see article above).
Males and females are affected equally; most patients are middle-aged.
Clinical Features [4]
Symptoms usually develop over days to weeks.
Symptom onset may be anywhere from two days to 18 months after exposure to gadolinium.
Skin fibrosis in NSF may mimic scleroderma, with predominant peripheral extremity involvement. It may involve the trunk, but it spares the neck and face and lacks the presence of serologic markers seen in scleroderma. The fibrosis may initially appear as plaques. They often cause skin pain, myalgia, arthralgia, and contractures.
Approximately 5% of patients have a rapid and fulminant course that may result in death.
It is now known that fibrosis also affects other organs, such as liver, lungs, muscle, and heart, which may contribute to the increased mortality of patients with NSF [5].
Symptoms may improve with improvement of renal function.