Nephrogenic Systemic Fibrosis Research Yields Few Clues Into Devastating Disease

Since 1997, medical science has been trying to solve the mystery of Nephrogenic Systemic Fibrosis (NSF). NSF, also known as Nephrogenic Fibrosing Dermopathy, is a rare and little understood disease that affects people with kidney problems. Since the first cases were documented, research into NSF has provided few clues into its cause, and even fewer as to its treatment

Prior to 1997, no cases of NSF had ever been reported. While it only appears in patients with pre-existing kidney problems, according to The International Center for Nephrogenic Fibrosing Dermopathy Research, there is no evidence that NSF is caused by kidney disease, medications, microorganism or dialysis. But in 2006, NSF researchers made an intriguing discovery. It was at that time that researchers linked the use of <"">Gadolinium based contrast agents used in MRIs with the development of NSF. Following the 2006 discovery, the Food and Drug Administration (FDA) warned healthcare professionals that Gadolinium based agents had been tied to multiple cases of NSF. Then, in 2007, the FDA went even further and requested that the manufacturers of the five Gadolinium based contrast agents used in MRIs include a boxed warning on product labels highlighting the risk they posed to patients with kidney problems.

Most patients eventually diagnosed with NSF are first found to have high blood pressure. That is followed by burning, itching, swelling and hardening of the skin. Other symptoms include red or dark patches on the skin; pain deep in the hip bones or ribs and muscle weakness. NSF can progress to the point of causing severe stiffness in joints, and it can lead to death. The progression of the disease can be slow, and it can take several years for all of the symptoms associated with NSF to appear. However, some patients have a form of NSF that progresses so quickly that they often die of complications brought on by the disease.

There are no consistently successful treatments for NSF, but symptoms can be improved with several methods. Physical therapy, oral steroids and some other drug therapies have shown promise in treating NSF. Unfortunately, NSF is such a recent phenomenon that few studies on treatments have been completed. According to the International Center for Nephrogenic Fibrosing Dermopathy Research, NSF patients show the most improvement when improved kidney function can ease symptoms, and in some patients this seems to reverse the disease overtime. Some NSF patients who have received kidney transplants have improved dramatically, but there is no guarantee that all patients will see such results.

While medical science works to unravel the mystery surrounding NSF, it is important that people with kidney problems be aware that the disease is associated with Gadolinium based contrast agents used in MRIs. These agents are injected into patients prior to an MRI in order to enhance images. Anyone with a kidney problem who is to undergo an MRI should make sure that agents with Gadolinium will not be used. Also, patients scheduled for an MRI using such contrast agents should always be screened for kidney

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