title: Pathomechanisms of nephrogenic systemic fibrosis: new insights.
abstract: Nephrogenic systemic fibrosis (NSF), previously known as nephrogenic fibrosing dermopathy, is a generalized fibrotic disorder occurring in people with renal failure, following exposure to gadolinium-based contrast agents used to enhance MRI. The cellular elements involved in pathology of NSF include bone-marrow-derived collagen-producing fibrocytes, myofibroblasts and activated macrophages. Mechanisms that have been hypothesized to play a role in the pathogenesis of NSF include upregulation of osteopontin, imbalance between matrix metalloproteinases and tissue inhibitor of metalloproteinase 1, and presence of transforming growth factor-β, nuclear factor κB, decorin and metallothioneins. Gadolinium (both free and chelated) is thought to be a bioactive trigger for NSF. Elucidation of these potential pathomechanisms would be useful for development of targeted therapies for NSF.
authors.affiliation: Division of Nephrology, University of Ottawa, Ottawa, Canada. parthankur@yahoo.com
authors.forename: A
authors.initials: A
authors.lastname: Gupta
authors.forename: M K
authors.initials: MK
authors.lastname: Shamseddin
authors.forename: A
authors.initials: A
authors.lastname: Khaira
mesh_terms: D003287:Contrast Media / Q000009:adverse effects*; D005682:Gadolinium / Q000009:adverse effects*; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000009:adverse effects; D054989:Nephrogenic Fibrosing Dermopathy / Q000139:chemically induced* / Q000378:metabolism / Q000503:physiopathology*
chemical_list: D003287:Contrast Media; D005682:Gadolinium