New Treatment Shows Promise for NSF Patients

Scott Kappes

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Nephrogenic Systemic Fibrosis is a rare fibrosing disorder that develops in the context of renal insufficiency. This extremely debilitating and painful disease has been strongly linked to the use of gadolinium contrast agents that are used to enhance the clarity of magnetic resonance imaging (MRI). NSF causes the infected persons skin to become rigid and thick, and can often leave them completely disabled and in need of around-the-clock attention. No treatment has been consistently effective in treating NSF but a new experimental treatment option, known as Extracorporeal Photopheresis, has shown promising results for three patients that have received the treatment.

Extracorporeal Photopheresis is form of dialysis that involves treating the blood with photoactive drugs that are then activated when the blood is exposed to ultraviolet light. The blood is then reintroduced to the body, ideally allowing the patient’s immune system to better combat the effects of NSF.

All three showed clinical response with softening of skin plaques at the end of four cycles, and improved range of movement in all four limbs on completion of treatment. The first patient, with NFD/NSF of 4.5 year's duration, was able to resume most activities of daily living although still chairbound, while a second patient with more recent onset of NSF was able to walk short distances with the help of Zimmer frames by the end of 16-18 cycles of treatment, having been chairbound pretreatment. A third patient with milder initial symptoms also experienced significant improvement after a shorter course of ECP treatment. Our experience with these three patients confirms previous case reports, suggesting that ECP may be effective in NFD/NSF.

While this treatment is still in its infancy as treatment for NSF it has shown very promising results as of thus far and offers bright hopes for the future in treating patients that have been crippled by this truly terrible disease.