Renal Transplantation Is Associated with Improved Clinical Outcomes in Nephrogenic Systemic Fibrosis

Abstract

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.