title: Nephrogenic systemic fibrosis: a systemic fibrosing disease resulting from gadolinium exposure.
abstract: Nephrogenic systemic fibrosis (NSF) is an iatrogenic fibrosing disorder that primarily affects individuals with chronic kidney disease (CKD) following exposure to gadolinium-based contrast agents (GBCAs) during imaging procedures. NSF is characterised by skin thickening, tethering and hyperpigmentation; flexion contractures of joints; and extracutaneous fibrosis. This article reviews the history, clinical manifestations, epidemiology, histopathology and pathophysiology of this disabling disease.
authors.affiliation: Rheumatology Division, Hospital for Special Surgery, and Department of Medicine, New York-Presbyterian Hospital/Weill Cornell Medical College New York, NY 10021, USA.
authors.forename: Elana J
authors.initials: EJ
authors.lastname: Bernstein
authors.forename: Christian
authors.initials: C
authors.lastname: Schmidt-Lauber
authors.forename: Jonathan
authors.initials: J
authors.lastname: Kay
mesh_terms: D003286:Contracture / Q000139:chemically induced; D003287:Contrast Media / Q000009:adverse effects* / Q000737:chemistry; D003937:Diagnosis, Differential; D005682:Gadolinium / Q000009:adverse effects* / Q000737:chemistry; D006801:Humans; D017495:Hyperpigmentation / Q000139:chemically induced; D054989:Nephrogenic Fibrosing Dermopathy / Q000139:chemically induced* / Q000150:complications / Q000175:diagnosis / Q000473:pathology / Q000628:therapy; D051436:Renal Insufficiency, Chronic / Q000150:complications*; D012307:Risk Factors; D012867:Skin / Q000473:pathology
chemical_list: D003287:Contrast Media; D005682:Gadolinium