Nephrogenic Systemic Fibrosis

Current treatments

NSF is usually a chronic, progressive condition. Rare cases of partial-to-complete spontaneous resolution have been reported. These resolutions have resulted not from a particular treatment, but in response to improvements in the patient's kidney function, either spontaneous or brought about by other treatment.

At this time there is no treatment for NSF that has been found to consistently effective for all patients. Dermatologists and other treating physicians have reported a variety of treatments, each of which has helped some sufferers. The following treatments and outcomes were reported in a 2006 paper by Noah Scheinfeld MD JD of Columbia University College of Physicians and Surgeons, and Shawn Cowper MD of Yale University.

• A patient for whom photopheresis resulted in some improvement in skin texture.
• The successful treatment of NFD with photodynamic therapy in a kidney transplant recipient.
• Several case reports in which Ultraviolet phototherapy sometimes improves NFD.
• the case of a 40-year-old patient with renal insufficiency who was treated with hemodialysis and who had undergone kidney transplantation. Two years late she developed sclerodermiform brownish plaques on her extremities. The condition improved significantly after 4 cycles of extracorporeal photopheresis.
• A case of NFD which responded to high-dose intravenous immunoglobulin.
• Oral prednisone has been effective in several cases, but it has been discontinued in some patients because of adverse side effects
• In 1 of 2 patients intralesional alpha interferon improved the skin, although in both patients, it had to be discontinued because of adverse side effects. In another patient, this therapy was associated with a worsening of lesions.
• A small series of patients with dual liver/kidney transplants showed marked improvement with plasmapheresis. Kidney function had improved post transplant, and the contribution of plasmapheresis to the improvement of NSF is unclear
• Topical calcipotriene (Dovonex) resulted in subjective improvement in 2 patients.

Research findings

In the 10 years since the first paper identifying NSF was published, much of the medical publication about the disorder has focused on describing it: the symptoms, the course of the disease, and attempts to apply existing treatments. Other clinical reports have ruled out other possible causes and confirmed the correlation between GBCA and the onset of NSF.

Basic research is now under way to identify the causal mechanisms of NSF: how the presence of gadolinium in people with severe kidney insufficiency or failure works at a molecular level to bring about the disorder. Learning the disease mechanism is the essential next step, because a treatment must be designed to interfere with one or more steps in that mechanism. Although recent research efforts have begun to shed light on these questions, the authors make clear that their findings are a beginning, and that more work is needed to establish a mechanism and devise a treatment.

"Nephrogenic Systemic Fibrosis", Noah Scheinfeld MD JD FAAD and Shawn Cowper MD, published May 2007, is a state of the art review of what is currently known about the disorder.

Website of the NSF Registry, maintained by Dr Cowper at http://www.icnfdr.org includes a summary of 34 papers published between 2000 and 2004. The papers cover all aspects of the disorder, and document the emerging understanding of Gadolinium Based Contrast Agents involvement.

"Gadolinium Deposition in Nephrogenic Fibrosing Dermopathy" Alan S. Boyd, John A. Zic, Jerrold L. Abraham Journal of the American Academy of Dermatology January 2007 (Vol. 56, Issue 1, Pages 27-30). The authors propose a possible mechanism for the disorder.

"Transglutaminases: The Missing Link in Nephrogenic Systemic Fibrosis" Parsons, Amy C MD; Yosipovitch, Gil MD; Sheehan, Daniel J MD; Sangueza, Omar P MD ; Greenberg, Charles S MD Sane, David C MD, American Journal of Dermatopathology. 29(5):433-436, October 2007. The authors propose the involvement of transglutaminases, and recommend further research.

"Risk factors for developing gadolinium-induced nephrogenic systemic fibrosis", Peak, A.S. Sheller, A, Annals of Pharmacotherapy, 2007 ep;41(9):1481-5

Support groups for victims and families

To reach the active online support group, click here http://health.groups.yahoo.com/group/nfd_support/ . As the disorder becomes better known, it's likely that more support groups will develop. Be sure to ask the doctors who treat you for NSF about support groups in your area.