Research Suggests Cause of NSF, a Debilitating Skin Condition, in Dialysis Patients

Researchers at Wake Forest University Baptist Medical Center, working with colleagues at other institutions have just released the findings of research into why some patients who are in kidney failure also develop a condition known as nephrogenic systemic fibrosis (NSF)  that tightens and swells the skin and other organs, among them the lungs and the heart. NSF can make the skin, usually on the arms and legs, to become rough or hard and can cause it to thicken so much that the limbs can become difficult or even impossible to move.

They have known that some patients, about 2 to 4%, who are on dialysis and are injected with a contrast agent, gadolinium by name, when they are given and MRI develop the disease. As a matter of fact, the U.S. Food and Drug Administration now requires a warning on the product labels warning of just this danger.

While they know that the drugs cause the problem they have, until now, not known how it does it. This study suggests that an enzyme named transglutaminase-2, or TG2 for short, may play a part.

During the course of their research, they tested the theory that TG2 may be involved. It is found throughout the body and is known to be involved in both the clotting of the blood and in healing wounds. The thought is that gadolinium may activate the enzyme and cause the development of NSF.

They obtained skin biopsies from 5 patients who had NSF and also from 3 heathy subjects. All of the NSF patients had renal failure and had had an MRI done where gadolinium was administered. They tested all the samples for the presence of TG2

The patients with NSF had a much higher level of TG2.
This told them that the activation of TG2 can produce the syndrom. Since TG2 is found in all tissues, it may explain the reason why the heart and lungs are also affected.

They also were able to suggest a way to prevent, or if necessary treat, NSF. There are drugs on the market already - such as cysteamine- that inhibit the activation of TG2.

This is a pilot study, but they believe that the results are a good basis for further research into the effect of TG2 not only in the dialysis, but also in other diseased that involve fibroids, or thickening of the skin.