title: Nephrogenic systemic fibrosis: clinicopathological definition and workup recommendations.
abstract: BACKGROUND
  The condition that came to be known as nephrogenic systemic fibrosis (NSF) was first reported in 2000 and, in 2001, was termed "nephrogenic fibrosing dermopathy." Since then, NSF has been the subject of a wide-ranging multidisciplinary medical investigation that has proven an indisputable link to renal disease and a compelling association with the increasing use of gadolinium-containing magnetic resonance imaging contrast agents in the renally impaired.
  
  
  OBJECTIVE
  Although precise causation and risk factors continue to be elucidated, the need for reproducible prospective epidemiologic data demands clear and objective criteria for the diagnosis of NSF.
  
  
  METHODS
  Experts in NSF diagnosis used their experience and the resources of the Yale International NSF Registry to develop a clinicopathological diagnostic system for NSF.
  
  
  RESULTS
  A consensus scoring system incorporating a clinical and histopathological atlas was devised to guide and standardize the evaluation and diagnosis of NSF.
  
  
  CONCLUSIONS
  There is no laboratory test that can be used as a gold standard to diagnose NSF. To overcome this, we relied on classic clinicopathological presentations, published sources, and consensus clinical expertise to ensure the integrity of the study population.
  
  
  CONCLUSIONS
  The clinicopathological definition of NSF provides guidance to physicians for the evaluation and diagnosis of NSF. Clinical, laboratory, and histopathological features comprise a schema that excludes conditions mimicking NSF while facilitating its reproducible and accurate diagnosis, even among physicians with little prior clinical experience with this entity. This definition can serve as a working diagnostic standard for future research and as the basis for adjudicating borderline cases.
authors: Department of Dermatology, Yale University School of Medicine, New Haven, Connecticut, USA.
authors: Michael
authors: M
authors: Girardi
authors: Jonathan
authors: J
authors: Kay
authors: Dirk M
authors: DM
authors: Elston
authors: Philip E
authors: PE
authors: Leboit
authors: Ali
authors: A
authors: Abu-Alfa
authors: Shawn E
authors: SE
authors: Cowper
mesh_terms: D006801:Humans; D054989:Nephrogenic Fibrosing Dermopathy / Q000175:diagnosis* / Q000209:etiology; D017410:Practice Guidelines as Topic