Nephrogenic Systemic Fibrosis, or NSF, is also known as Nephrogenic Fibrosing Dermopathy, or NFD, is a rare and serious disease that affects only patients with pre-existing kidney problems. NSF involves fibrosis of skin, joints, eyes, and internal organs where scar tissue forms that is so thick the skin and joints become immobile and this appears to be associated with Gadolinium exposure and people with severe kidney failure. Patients with NSF will develop large areas of hardened skin due to fibrotic nodules and plaques and shows a proliferation to the dermal fibroblasts and dendritic cells, increased elastic fibers, thickened collagen bundles, and deposits of mucin, and in some more serious cases, NSF can paralyze the internal organs ending with fatal results.
The first case of NSF appeared in patients around 1997, displaying symptoms such as high blood pressure, burning and itching, and swelling or hardening of the skin, but was labeled as an independent disease. Some other symptoms that also appear in patients with NSF include red or dark patches on the skin, muscle weakness, and pain that can be felt deep in the hip bones or ribs. NSF may even progress to the point of severe stiffness of the joints and can even be so serious and dangerous to lead to death.
NSF was discovered in 2006 as having a direct link relating the use of Gadolinium Magnetic Resonance Imaging (MRI) contrast dyes in patients with kidney problems. As a result of multiple studies showing this same association, later in 2006, the Food and Drug Administration issued a notification addressed to health care providers and the general public stating that patients with moderate to end-stage kidney disease that may receive a MRI or MRA where the Gadolinium based contrast dye may develop Nephrogenic Systemic Fibrosis, or NSF. Then in 2007, the FDA decided that manufactures of Gadolinium contrast dyes to place a black box warning, which is also the strictest type of FDA warning, on the product labels to advise people with kidney problems of the risk of NSF.
Currently, there is not any cure for NSF, and the specific cause is still not completely understood. Yet, the evidence of the role that Gadolinium MRI contrast may play in the development of NSF is steadily becoming irrefutable. Researchers at Yale University have reported that 95 percent of those with NSF had an MRI that involved a Gadolinium MRI contrast dye two to three months before the symptoms appeared. Another study done by researchers at Massachusetts General Hospital has also found that kidney patients who had undergone MRIs with Gadolinium MRI contrast dyes were ten times more likely to develop NSF than patients who had not been exposed to such agents.
What this means for patients with NSF is that life can become very unbearable as the skin becomes so hardened, the joints become so stiff that movement becomes impossible, and the disease may even start affecting the heart, lungs and liver. NSF can even lead to death as there are not any effectively known treatments; however, some patients have shown improvement after receiving a kidney transplant.
As it is, the FDA is urging all patients that may believe they suffer from NSF to immediately contact their doctor. Should a patient with kidney disease need to have an MRI or MRA done, the FDA is also encouraging those patients to seek other imaging methods that do not use the Gadolinium based contrast whenever possible as there is currently no alternative for Gadolinium based MRI contrast agents.
If a patient has found that they have developed NSF after receiving a MRI or MRA, then please click here to have a free case evaluation done.