Diagnosis and reporting grid for nephrogenic systemic fibrosis (NSF) scoring system

Using grid:
Clinical score is located across top row of grid, whereas histologic score is located along left vertical column. Intersection of these 2 values within chart results in 1 of 6 diagnostic possibilities. For epidemiologic purposes, those in green should be scored as confirmed nephrogenic systemic fibrosis (NSF) cases. Those in yellow should be closely re-examined and perhaps rebiopsied. Until more compelling evidence is obtained, these will not have satisfied criteria for NSF and should be considered nondiagnostic of NSF. Those in red are ruled out either for lack of criteria or because another Dx can be made.

Caveats:

1. Absence of documentable kidney disease: In the unlikely event the final patient score falls into NSF box, yet current or prior kidney disease cannot be established, the most certain Dx that should be rendered is "suggestive of NSF." Consultants with extensive NSF experience should evaluate any such case, as it would represent a highly unusual occurrence.
2. Postmortem evaluation of suspected NSF: The histologic assessment of NSF in the postmortem setting is not difficult, provided the Dx is suspected clinically and generous samples of contiguous skin, fascia, and underlying muscle are obtained. This type of specimen is not gathered during a routine postmortem examination, however, so the pathologist must be made aware of the suspected diagnosis in advance to assure appropriate sampling. The clinical examination is much more challenging as neither induration nor range of motion can be appropriately assessed, and livedoid patterning may occur as a normal postmortem finding. Scleral plaques in persons younger than 45 years of age, if combined with excellent histology and a good physician-documented corroborative medical record, may enable a definitive Dx in the postmortem setting.