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