Nephrogenic Systemic Fibrosis Signs and Symptoms

Nephrogenic Systemic Fibrosis (NSF) is a painful and rare but potentially life threatening disease of the internal organs and skin. First recognized in the 1997, it is found only in few patients – those who suffer from a serious kidney problem or those who have undergone magnetic resonance angiography (MRA) or magnetic resonance imaging (MRI) tests. In fact, according to one study, above percent of the patients suffering from NSF were exposed to some or the other type of gadolinium within the four months of developing this disease. In this response, the United States Food and Drug Administration asked, doctors and/ or medical health care professionals to avoid using gadolinium in MRA/ MRI in general and in patients suffering from kidney failure. Researchers and scientists in the Europe and United States are continually working to determine whether to ban this substance altogether or not.

According to scientists, there is high possibility that gadolinium may cause Nephrogenic Systemic Fibrosis it reacts badly with high level of acid present in the body. As the patients suffering from kidney failure fail to process impurities out of their body and healthy people, they have higher level of acids in their bodies, a condition called acidosis. The brand names of gadolinium-containing agents or dyes approved to be used in the United States in MRIs include the following:

ProHance

Omniscan

Magnevist

OptiMARK

MultiHance

Brand names of gadolinium-containing agents or dyes that are used in other countries include Vasovist, Gadovist, Dotarim and Primovist.

Debilitating and Painful Symptoms:

Originally, Nephrogenic Systemic Fibrosis was called Nephrogenic Fibrosing Dermopathy, because of its most obvious and first symptoms include rough patches or lesions on the skin of the patient. After a period of few days to week, patients start noticing a tightening and selling of the skin, particularly the skin on the hands, feet, arms and legs. Often, these patches occur in symmetry meaning that a spot on the patients left arm will appear in a similar place on the right arm. Soon, the skin develops lesions – areas where it gets darker or redder which itch, burn or send sharp pains in that area. On a microscopic level, researchers and scientists discovered that these lesions occur due to build up of connective tissues and skin – especially scar tissue.

Over the period of short time, these lesions become woody and hard, resembling just like an orange peel. Eventually, due to this hardening, patients fail to bend their knees, fingers, elbows or other joints – robbing patients of their ability to perform daily tasks, to walk or even care for themselves. And this disease attacks the other organs of the patients’ body in the similar way, making a buildup of scar tissue that could ultimately stop the functioning of internal organs and may even lead to death.

In addition to these above listed symptoms, patients suffering from NSF may also report the following symptoms:

Sudden development of high blood pressure (hypertension)

Bone pain in the ribs and hips

Blister-like spots on the hands

Yellow lesions near or on the eyes

Muscle weakness

Because Nephrogenic Systemic Fibrosis is solely found in the patients suffering from kidney failure, often, its symptoms are disguised by the existing symptoms of this serious disease. In fact, according to some scientists, NSF remains under diagnosed in many kidney patients. Additionally, NSF is discovered about three weeks after the patient underwent a severe medical problem or surgery, like the failure of transplanted kidney, angioplasty, deep vein thrombosis or any other traumatic event affecting the heart and the blood vessels. Currently, researchers and scientists are investigating whether these traumas lead to NSF or its effects. Many of them are diagnosed with MRA / MRI tests, suggesting that the test and the use of gadolinium could be one of the problems.

Crippling Effects:

Even if NSF fails to affect the patients’ independence, it would be immobilizing because of its disfiguring and painful effects on the skin. However, as it also causes physical inflexibility, muscle weakness and joint pain in the patients who already suffers from serious kidney problems, NSF can be highly crippling, placing some patients in the wheel chair within few weeks of diagnosis. In extreme cases, patients need the help of others to care for them, to move and perform the daily tasks of living. For many people who have fought hard to live their lives independently after undergoing renal failure, this could be emotionally devastating too. And Nephrogenic Systemic Fibrosis presents a real threat of death; directly through the build up of scar tissue on important organs and indirectly through accidents and falls caused due to the failure of patient’s mobility. That is really a high price to pay for a diagnostic test.

As of December 21, 2006, the United States federal Food and Drug Administration knew at least 210 cases of Nephrogenic Systemic Fibrosis worldwide. Of these 210 patients, all the patients whose earlier medical histories were reviewed revealed that they were exposed to gadolinium in an MRA or MRI test. Additionally, tests found the presence of gadolinium in the skin lesions of the patients suffering from NSF. Although more research and studies are needed to substantiate these findings, this strong correlation between NSF and gadolinium has led to the lawsuits in the entire country. As researchers start to confirm this link between gadolinium and Nephrogenic Systemic Fibrosis, observers expect more cases of NSF.