Nephrogenic Systemic Fibrosis

As if the world weren’t dangerous enough new diseases keeping popping up like, well new diseases. Over the past few years nephrologists have recognized a new skin disease that was previously called nephrogenic fibrosing dermopathy, but which has been rechristened Nephrogenic Systemic Fibrosis. The current issue of the NEJM describes another patient with this disorder.

The disorder is only seen in patients with renal disease – typically in patients with renal failure on dialysis. The skin becomes tight and can so limit mobility that contractures develop. Fibrosis of internal organs has also been described. Recently an association with the use of gadolinium has been reported. Gadolinium is used as a contrast agent to enhance the images obtained with MRIs. How gadolinium might cause nephrogenic systemic fibrosis is not known. Most nephrologists currently avoid using the agent in patients with impaired renal function. While radiologists have set a glomerular filtration rate of 30 ml/min as the cut-off below which gadolinium should only be used for the most urgent problem, there’s no way right now of knowing exactly how decreased renal function must be for this potentially lethal disorder to occur. The disease is progressive and the mortality rate high. The best strategy is to prevent it.

Of course never at a loss for a business opportunity trial lawyers are advertising like trial lawyers to recruit patients who’ve developed nephrogenic systemic fibrosis after gadolinium exposure even though the association of the disease and the contrast agent was only first made in 2006. Put the disease into Google and the first listings will be paid ads by trial lawyers soliciting new plaintiffs.

After the lawyers comes The International Center for Nephrogenic Fibrosing Dermopathy Research. A support group inevitably will follow. Good intentions and profit seem inextricably linked.

Right now there’s no treatment for the disorder. Also unknown is how common it will turn out to be and what its range of severity will be. The best advice for patients with renal disease is to avoid gadolinium unless there is extraordinary need for it. How tight the association of this disease with the contrast agent is also uncertain.

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