Nephrogenic systemic fibrosis, also known as nephrogenic fibrosing dermopathy, is a debilitating disorder affecting the skin. The condition affects both men and women of every age, from infants to the elderly, but typically occurs in individuals that are middle aged. The condition can affect individuals of every ethnicity and has been found mainly in Europe, Asia, and North America. The condition is considered rare, with less than 300 confirmed cases worldwide.
Currently, all cases of NSF/NFD occur in patients in an advanced stage of kidney disease. None of the cases has occurred prior to 1997 and no evidence exists to link the condition to a microorganism, to an ingested medication, or to dialysis. The length of time that the individual has been suffering from kidney disease also appears to not be a factor in the development of NSF/NFD as it can occur in the earlier stages of kidney disease as well as in individuals that have been suffering from kidney disease for years.
Recent reports have found a strong correlation between the development of NSF/NFD and drugs containing gadolinium which are used as contrast agents during an MRI. By December of 2006, the US Food and Drug Administration had received 90 reports of cases of NSF/NFD occurring in patients that had recently received an injection of a gadolinium based contrast agent prior to undergoing an MRI. Of the cases studied in depth by the US Food and Drug Administration, all of the patients were in moderate to late stage kidney disease, all had received an injection of a gadolinium based contrast agent, and all had undergone an MRI.
Individuals experiencing NSF/NFD have reported the skin of their extremities swell and tighten, limiting movement and causing great discomfort. In rare cases, the condition affects the trunk of the body as well. Patients with severe cases of the condition may be unable to stretch and flex their arms, hands, legs, or feet and may even lose the ability to walk. The individual may experience significant muscle weakness and moderate to severe joint pain. Bone pain occurring in the hips and ribs are also commonly reported symptoms of NSF/NFD.
Around 5% of cases of NSF/NFD progress rapidly, quickly changing the skin from supple to thick and stiff within a period of months. The condition starts with reddened or dark patches appearing on the skin, accompanied by an itching, burning sensation. As the condition progresses, the texture of the skin starts to resemble the peel of an orange and radiography will reveal that the soft tissue of the skin has undergone calcification. Areas affected by NSF/NFD become hard and stiff and severe sharp pains may occur in the tissue beneath the affected skin.
There is no effective treatment option available for NSF/NFD, but the symptoms of the condition can be relieved with numerous treatments. Improvement of the renal function of the patient has the ability to slow, halt, or even reverse the progression of NSF/NFD. Because the condition is rare and was discovered relatively recently, there are a lot of things about the condition that are not understood. Research continues to help medical professionals understand and conquer this debilitating disease.
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