Treatment Options for Nephrogenic Systemic Fibrosis / Nephrogenic Fibrosing Dermopathy

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Nephrogenic systemic fibrosis (NSF) is an extremely rare condition that occurs in some individuals enduring an advanced stage of kidney disease. Also known as nephrogenic fibrosing dermopathy (NFD), the first cases of the condition were documented in 1997. Since then, less that 300 cases have been confirmed worldwide. Researchers are unsure what causes the condition, but recent studies have indicated that a connection exists between NSF/NFD and solutions commonly used in MRI procedures called Gadolinium based contrast agents. To date, the US Food and Drug Administration has received over 90 reports of NSF/NFD cases linked to the injection of a Gadolinium based contrast agent.

NSF/NFD is a skin disorder characterized by the skin located on the extremities becoming tight and stiff to the point of extreme discomfort. The surface of the skin begins to resemble orange peel and the soft tissue beneath the affected areas begins to harden. Patients afflicted with NSF/NFD report that they feel an itching, burning sensation in the skin and sometimes experience sharp, shooting pains within the tissue underneath the affected areas. The condition typically develops over a period of months, but in some cases, the condition can go from non-existent to severe within a matter of days.

There is no one recommended treatment option for NSF/NFD that is consistently effective, although there are quite a few treatment options that are commonly used to alleviate the symptoms of the condition. One commonly used treatment method is the prescription of oral steroids, namely prednisone. This oral steroid is used in the treatment of a large number of different conditions and has been shown to be effective in a number of inflammatory diseases. The medication has also been shown to improve renal function in patients with kidney disease so NSF/NFD is being battled on both fronts.

Another treatment option used to relieve the symptoms of NSF/NFD is Dovonex. Dovonex is a topical medication that can improve the skin sensations experienced by individuals suffering from NSF/NFD. This medication is derived from naturally occurring vitamin D and is not considered a steroid like many other topical medications. Dovonex can significantly decrease the irritation, redness, and scaling signature to NSF/NFD but it cannot cure the condition. The medication can cause irritation if placed on the face, so patients are warned to avoid accidental transfer to the facial area.

Some individuals with NSF/NFD have found that regular physical therapy has been effective at slowing the stiffening of the joints. Activities such as swimming can help keep the joints mobile while avoiding the jerky movements that can cause joint damage. Deep massage has also been shown to be effective in relieving some of the pain associated with NSF/NFD. Many physicians recommend that regardless of which other treatments are pursued, physical therapy and deep massage should be included in the treatment regiment.

There are quite a few other treatments available for the relief of symptoms of NSF/NFD. Each works with varying degrees of success, but none are effective all the time with every patient. Researchers continue to try to find an effective treatment and a cure for NSF/NFD, but any breakthroughs in those areas are still years away.

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