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UV-A1 therapy for nephrogenic systemic fibrosis
Kien T Tran1, Heidi B Prather, Clay J Cockerell
1Department of Dermatology, University of Texas-Southwestern Medical Center at Dallas, Dallas, TX 75390-9069, USA.
Background:
Nephrogenic systemic fibrosis (NSF) is a rare sclerosing skin condition associated with end-stage renal disease and gadolinium exposure. Therapy for NSF is challenging, with few options other than preventing exposure to gadolinium and improving renal function through transplant. However, in some cases neither of these options is tenable. We report the successful use of UV-A1 phototherapy in 4 patients with NSF.
Observations:
Four patients with NSF were treated with UV-A1 phototherapy at a tertiary medical center from 2005 through 2007. To our knowledge, it is unique to this series that all patients were receiving hemodialysis before, during, and after therapy with UV-A1. All experienced improvement in the degree of induration, and 2 experienced improvement in mobility of the hands and legs. Total treatments ranged from 22 treatments (with a cumulative dose of 1855 J/cm(2)) to 50 treatments (total UV-A1 exposure, 3850 J/cm(2)). No adverse events were observed.
Conclusions:
Although no patient had complete resolution of indurated plaques, the improvement was substantial. For 2 patients, it resulted in a resumption of hand and leg mobility. As a result, UV-A1 therapy may represent a treatment for NSF when kidney transplantation is not an option or is delayed. Limitations of this study include the lack of a controlled trial, lack of quantification of gadolinium levels within tissue, and the lack of a defined grading scale for NSF severity.
Insights
UV-A1 phototherapy shows promise for treating Nephrogenic Systemic Fibrosis (NSF), a rare skin condition. This treatment improved skin induration and mobility in patients with end-stage renal disease when transplantation was not an option.
Area of Science:
- Dermatology
- Nephrology
Background:
- Nephrogenic Systemic Fibrosis (NSF) is a rare, debilitating skin condition linked to end-stage renal disease and gadolinium exposure.
- Current therapeutic options for NSF are limited, primarily focusing on gadolinium avoidance and renal function improvement via transplantation.
- In some cases, transplantation is not feasible, necessitating alternative treatment strategies.
Purpose of the Study:
- To evaluate the efficacy and safety of UV-A1 phototherapy as a treatment for Nephrogenic Systemic Fibrosis (NSF).
- To assess the impact of UV-A1 phototherapy on skin induration and patient mobility in NSF patients undergoing hemodialysis.
Main Methods:
- A case series involving four patients diagnosed with NSF who received UV-A1 phototherapy between 2005 and 2007.
- All participating patients remained on hemodialysis throughout the UV-A1 treatment period.
- Treatment duration varied from 22 to 50 sessions, with cumulative UV-A1 doses ranging from 1855 J/cm(2) to 3850 J/cm(2).
Main Results:
- All four patients demonstrated improvement in the degree of skin induration.
- Two patients experienced enhanced mobility in their hands and legs following UV-A1 therapy.
- No adverse events were reported during the course of the phototherapy treatments.
Conclusions:
- UV-A1 phototherapy offers a potential treatment option for NSF, particularly when kidney transplantation is not viable or is delayed.
- While complete resolution of NSF plaques was not achieved, substantial clinical improvement was observed.
- Further research, including controlled trials and standardized severity grading, is warranted to fully establish UV-A1 therapy's role in NSF management.
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