Nephrogenic systemic fibrosis

Abstract

Nephrogenic systemic fibrosis is a recently diagnosed disease that occurs in patients with chronic kidney disease and acute renal failure. The patients develop skin thickening and fibrosis which is usually symmetrical, and typically of the upper and lower extremities. In some cases the progression is rapid leading to joint contractures confining the patient to a wheelchair. Systemic involvement may occur, leading to cardiomyopathy, pulmonary fibrosis, pulmonary hypertension, diaphragmatic paralysis and in severe cases death. The pathophysiology of the disease still remains unclear, but recent studies have demonstrated gadolinium deposits in tissues of patients diagnosed with nephrogenic systemic fibrosis. The prevalence of nephrogenic systemic fibrosis after exposure to gadolinium has been reported to be up to 12% in CKD stage 5 patients after a single exposure. No single treatment has been shown to be effective, although there are some patients shown to have improvement of their clinical symptoms with regaining of renal function especially after transplantation. In this article we review the current literature of this disease. Copyright © 2008 S. Karger AG.