Treatments for Nephrogenic Systemic Fibrosis (NSF)

There is still no cure for Nephrogenic Systemic Fibrosis (NSF). Likewise, there is no proven treatment for its symptoms. However, results of some recent clinical trials are promising, with NSF victims getting partial relief from their symptoms.

UV-A1 Phototherapy

A recent study has shown that UV-A1 phototherapy can reduce symptoms of NSF.  UVA radiation has a long wavelength that enables it to reach the subcutis (the connective tissue beneath the skin).

In a study conducted by the Dermatology Department of the University of Texas Southwestern Medical Center in Dallas, four NSF patients were treated with a UV-A1 bed.  After treatment, the effected areas of the skin on all four of the patients became less hard, and had decreased pain and itching.  Also, two patients had increased joint mobility.  The patients were tracked for varying lengths of time after treatment (six months to one year), and their improved skin condition and mobility lasted during those periods.  This is significant, since the improvements from other treatments have not been shown to last after treatment ends.

For more information, see Tran, et al., “UV-A1 Therapy for Nephrogenic Systemic Fibrosis,” Archives of Dermatology, Vol. 145, No. 10, Oct. 2009, pp. 1170-1182.

Gleevec, Generic Name Imatinib Mesylate

Recent studies indicate that cancer drug Gleevec, generic name Imatinib Mesylate (IM), may lessen the skin symptoms of Nephrogenic Systemic Fibrosis (NSF).

IM, marketed by Novartis as Gleevec in the United States, and Glivec in Europe, is a drug used for treating chronic myelogenous leukemia, gastrointestinal stromal tumors, and a number of other cancers. The FDA approved IM for cancer treatment in 2001.

IM was first reported as a possible treatment for NSF in 2008, in an article by Drs. Jonathan Kay and Whitney A. High. See “ Imatinib Mesylate Treatment of Nephrogenic Systemic Fibrosis, Arthritis & Rheumatism 2008; 58(8): 2543-48. Two patients with stage 5 chronic kidney disease and NSF were treated with 400 mg. of IM per day. Skin thickening and skin tethering decreased in both patients, followed by increased knee extension. However, when they stopped taking the drug, their symptoms returned. Skin biopsies performed before and after treatment revealed less fibrosis, but the same amount of gadolinium in their skin.

Dr. Kay has continued research on IM as a treatment for NSF, recently completing a clinical trial. This recent study yielded similar results, this time in six NSF patients treated with 400 mg. of IM per day over a four-month period. See Cancer Drug Gleevec Shows Promise for Treatment of Nephrogenic Systemic Fibrosis (NSF).

Rapamycin, also known as Sirolimus

As we noted in a recent blog entry, Rapamycin is an immunosuppressant drug that may improve symptoms of NSF. Some NSF patients have seen their symptoms improve or progression of the disease slow after a kidney transplant. That may be the result of the immunosuppressant drugs used in conjunction with the transplant procedure.

A Case Report in the February 2010 Journal of the American Academy of Dermatology reports that an NSF patient treated with rapamycin for three weeks experienced a “dramatic” decrease of swelling, hardening and pain in his lower legs. The authors believe this is because tissue effected by NSF contains a protein “downstream” from the protein targeted by rapamycin. For more information, see Swaminathan, et al., “Rapid Improvement of Nephrogenic Systemic Fibrosis with Rapamycin Therapy: Possible Role of Phospho-70-ribosomal-S6 kinase,” Journal of the American Academy of Dermatology, Vol. 62, Issue 2, pp. 343-45 (Feb. 2010).

Extracorporeal Photopheresis

Extracorporeal photopheresis (ECP), or simply photopheresis, is a dialysis-like procedure in which blood is first removed from the body, then treated with ultraviolet light and drugs that become active when exposed to light. The blood is then returned to the body. The treatment is currently only approved by the FDA for treatment of T-cell lymphoma.

A 2007 study at Stanford University reported some improvement of NSF symptoms from this procedure. Five NSF patients were given an average of 34 extracorporeal photopheresis (ECP) treatments over a period lasting, on average, eight-and-one-half months. Three of the five patients experienced mild improvement in skin tightening, range of motion, and/or functional capacity. See Richmond, et al., “ Nephrogenic Systemic Fibrosis; Relationship to Gadolinium and Response to Photopheresis,” Arch. Dermatol. 2007; 143(8):1025-1030.

This treatment seems to work best for patients who have had NSF symptoms for less than a year.

If you or a loved one has symptoms of Nephrogenic Systemic Fibrosis (NSF), contact an NSF lawyer or NSF attorney at DeCarli Law for a free consultation.  You may have a legal claim against the manufacturer of the gadolinium-based contrast agent that caused your disease.  The above, experimental treatments are costly.  The manufacturers that caused your condition should be made to pay for your treatments.  Bringing an MRI side effect lawsuit, or gadolinium lawsuit, against the manufacturers, is the only practical way to force them to compensate you for your injury and provide you money to pay for your NSF treatment.