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Pathomechanisms of nephrogenic systemic fibrosis: new insights
Author(s) -
Gupta A.,
Shamseddin M. K.,
Khaira A.
Publication year - 2011
Publication title -
clinical and experimental dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.587
H-Index - 78
eISSN - 1365-2230
pISSN - 0307-6938
DOI - 10.1111/j.1365-2230.2011.04136.x
Subject(s) - nephrogenic systemic fibrosis , fibrocyte , fibrosis , medicine , osteopontin , pathogenesis , matrix metalloproteinase , pathology , myofibroblast , tissue inhibitor of metalloproteinase , gadolinium , immunology , chemistry , kidney disease , organic chemistry
Summary 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.