Nephrogenic systemic fibrosis

Abstract

Nephrogenic systemic fibrosis (NSF) is a debilitating fibrosing disorder that has been observed almost exclusively among patients with compromised renal function following exposure to gadolinium-containing contrast agents. It presents with brawny hyperpigmentation and tethering of skin, predominantly on the extremities, and fixed flexion contractures of the knees, ankles, elbows, and fingers. Yellow scleral plaques with capillary dilatation may be evident on the eyes and visceral involvement can cause significant morbidity and mortality. A deep skin biopsy demonstrating increased numbers of spindle-shaped fibroblast-like cells with dual CD34- and procollagen I-positive immunohistochemical staining in the dermis and thickened collagen bundles separated by adjacent clefts confirms the diagnosis of nephrogenic systemic fibrosis in a patient with characteristic clinical features. Treatment with tyrosine kinase inhibitors may reverse existing fibrosis and prevent progression of disease.