title: [Nephrogenic fibrosing dermopathy or nephrogenic systemic fibrosis? What do we know and what do we have to learn?].
abstract: Nephrogenic systemic fibrosis (NSF) was first recognized as a unique entity in 1997 and subsequently defined in the literature in 2000 as a novel fibrosing disorder occurring in the setting of renal disease. Prevention, early recognition and treatment are essential to limiting its impact. The most important risk factors for developing NSF are chronic or significant acute kidney disease (especially dialysis dependent patients) and the administration of gadolinium (GD3) containing contrast agents, agents that cause NSF by releasing free gadolinium (GD3) into tissues based on their pharmacokinetics. International commissions in drug control and medicinal products recommend to avoid gadolinium based contrast agents in patients with GFR < 30 ml/minute/1.73 m(2). Unfortunately there is lack of universally effective therapy at this time and the literature is based on case reports and small case series. Recommendations to guide the use of gadolinium based contrast agents in patients with underlying kidney disease should be individualized and considered in consultation with the ordering physician, radiologist and nephrologist.
authors.affiliation: Servicio de Nefrología e Hipertensión, Universidad de Miami, Miller School of Medicine, Miami, FL, USA. lortega2@med.miami.edu
authors.forename: L M
authors.initials: LM
authors.lastname: Ortega
authors.forename: G
authors.initials: G
authors.lastname: Contreras
authors.forename: O
authors.initials: O
authors.lastname: Lenz
mesh_terms: D002614:Chelating Agents / Q000009:adverse effects; D000075202:Contraindications; D003287:Contrast Media / Q000009:adverse effects / Q000493:pharmacokinetics; D005347:Fibroblasts / Q000187:drug effects; D005682:Gadolinium / Q000009:adverse effects / Q000493:pharmacokinetics; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007677:Kidney Function Tests; D008279:Magnetic Resonance Imaging; D054989:Nephrogenic Fibrosing Dermopathy / Q000139:chemically induced / Q000209:etiology / Q000503:physiopathology / Q000517:prevention & control / Q000534:rehabilitation / Q000628:therapy; D010431:Pentoxifylline / Q000627:therapeutic use; D017893:Photopheresis; D026741:Physical Therapy Modalities; D010956:Plasmapheresis; D017410:Practice Guidelines as Topic; D006435:Renal Dialysis; D051437:Renal Insufficiency / Q000150:complications / Q000628:therapy; D012307:Risk Factors
chemical_list: D002614:Chelating Agents; D003287:Contrast Media; D005938:Glucocorticoids; D016756:Immunoglobulins, Intravenous; D005682:Gadolinium; D010431:Pentoxifylline