Nephrogenic systemic fibrosis: a clinicopathologic study of six cases.

Abstract

Nephrogenic systemic fibrosis is a rare fibrosing condition that occurs in patients with renal insufficiency. While its histologic characteristics have been well described, the etiology and pathogenesis have not been fully characterized. Several recent studies support the theory that gadolinium-based contrast agents play a causative role in the development of the disease. Erythropoietin therapy and endothelial damage from surgical procedures have also been suggested as potential contributing factors. This study attempts to help contribute to the understanding of this novel disorder. We performed a retrospective chart review of 6 patients diagnosed with nephrogenic systemic fibrosis at our institution. Emphasis was placed on identification of potential putative etiologic agents including gadolinium, erythropoietin therapy, and previous surgical procedures. All patients had documented exposure to gadolinium-based contrast agents. Three of the 6 patients were treated with erythropoietin, and all patients underwent a previous surgical procedure. This study is limited by its small size; therefore, the findings and results may not be applicable to all patients with this disorder. Our data suggest that gadolinium plays a primary role in nephrogenic systemic fibrosis and that prior surgery may be a contributory factor.