UV-A1 therapy for nephrogenic systemic fibrosis.
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.


METHODS
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.
Department of Dermatology, University of Texas-Southwestern Medical Center at Dallas, Dallas, TX 75390-9069, USA.
Kien T
KT
Tran
Heidi B
HB
Prather
Clay J
CJ
Cockerell
Heidi
H
Jacobe
D000368:Aged; D001707:Biopsy, Needle; D004307:Dose-Response Relationship, Radiation; D005260:Female; D005500:Follow-Up Studies; D005682:Gadolinium / Q000009:adverse effects; D006801:Humans; D007150:Immunohistochemistry; D007676:Kidney Failure, Chronic / Q000139:chemically induced / Q000150:complications / Q000473:pathology* / Q000628:therapy; D008875:Middle Aged; D054989:Nephrogenic Fibrosing Dermopathy / Q000139:chemically induced / Q000150:complications / Q000473:pathology* / Q000532:radiotherapy*; D011829:Radiation Dosage; D012008:Recurrence; D006435:Renal Dialysis / Q000379:methods; D018570:Risk Assessment; D012494:Sampling Studies; D012720:Severity of Illness Index; D013997:Time Factors; D016896:Treatment Outcome; D014467:Ultraviolet Therapy / Q000379:methods*
D005682:Gadolinium