Nephrogenic systemic fibrosis is a debilitating disorder seen in chronic kidney disease patients and is characterized by stiffening of the joints and thickening of the skin. Treatment options are limited, but some patients have had an improvement of their clinical symptoms after renal transplantation and the use of immunosuppression. Although there is a variable response to renal transplantation, it is currently unknown what factors promote a favorable outcome. Our objective was to evaluate if the response to renal transplantation was superior to other treatment modalities and to determine which characteristics allowed for a positive response to occur. We retrieved the data from the literature of 298 reported patients, compared the response to renal transplantation and to other treatments, and analyzed their characteristics. We found that more patients had a higher response to renal transplantation, as determined by softening of the skin and improved joint mobility, and among those that did respond, they had a shorter dialysis vintage. We suggest that if renal transplantation is to be considered as a treatment modality, it should be initiated at the earliest possible in the course of the disease to achieve maximum clinical benefit. 1. Introduction Nephrogenic systemic fibrosis (NSF) is primarily seen in chronic kidney disease (CKD) patients and is characterized by large areas of hardened skin with slightly raised plaques, papules, and hyperpigmentation. Biopsies of the affected areas show increased numbers of fibroblasts, alteration of the normal pattern of collagen bundles, and often increased dermal deposits of mucin [1]. The skin may have a “cobblestone” [2], “woody” [3, 4], or peau d’ orange appearance [5]. These lesions may be pruritic and accompanied by sharp pain or burning sensations [3, 6]. Movement of the joints may be so severely limited by the fibrosis that the flexibility is lost. The first cases were noted between 1997 and 2000 in hemodialysis (HD) patients or patients with a failed renal allograft who developed severe skin indurations that were initially thought to be scleromyxedema [7, 8]. Since Grobner reported a correlation between the use of gadolinium (Gd) in end-stage renal disease (ESRD) patients and NSF [9], additional reports supporting this correlation have been published [10, 11]. It is now recognized that low-stability gadolinium-based contrast agents, in the presence of permissive factors, most likely trigger the disease. The prevalence of NSF after exposure to gadodiamide (Omniscan) has been reported to be between 3% and 7%
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