Original article
Nephrogenic systemic fibrosis: Clinicopathological definition and workup recommendations

Elements of the draft version of this schematic were presented at the Third Annual Yale Symposium on Nephrogenic Systemic Fibrosis and Gadolinium-based Contrast Agents, New Haven, Connecticut, May 9, 2009.
https://doi.org/10.1016/j.jaad.2010.08.041Get rights and content

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.

Limitations

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.

Conclusion

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.

Section snippets

Overview

The core methodology was to divide the consideration of clinical and histopathological criteria between two sets of expert physicians (clinicians and pathologists). These teams were tasked with developing objective criteria for diagnosis and identifying exclusionary criteria based on the differential diagnosis of NSF. The teams identified criteria within their purview (drawing on professional experience, the medical literature, and the resources of the NSF registry3) and developed clinical and

Results

The results of this work consist of a recommended clinical and histopathological diagnostic evaluation for patients in whom NSF is suspected, a clinical and pathological scoring system keyed to an atlas of visual standards, and a diagnostic grid that combines the clinical and pathological scores to recommend a final diagnosis. These are detailed in Table IV, Table V, Table VII, Table VIII, Table IX, Table X, Table VI and Fig 1, Fig 2, Fig 3, Fig 4, Fig 5, Fig 6, Fig 7, Fig 8, Fig 9, Fig 10, Fig

Renal disease

See Table IV. Prevailing theories regarding the development of NSF are based on the observation that disease onset and activity depend on underlying renal dysfunction. This dysfunction could be characterized as a decreased rate of or absent glomerular filtration, either acutely or chronically. Because this concept is so powerfully ingrained in the medical literature about NSF, the authors concluded that any cases defined as “consistent with” or “diagnostic of” NSF without a known history of

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    Please visit http://www.eblue.org for the unabridged version of this manuscript.

    This project was coordinated by ACR Image Metrix, an American College of Radiology–owned company. Funding for the project was provided to ACR Image Metrix equally by the following organizations: Bayer HealthCare, Bracco, Covidien, and GE HealthCare.

    Conflicts of interest: None declared.

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