Nephrogenic systemic fibrosis: a clinicopathologic study of six cases.
BACKGROUND
Nephrogenic systemic fibrosis is a rare fibrosing condition that occurs in patients with renal insufficiency. While its histologic characteristics have been well described, the etiology and pathogenesis have not been fully characterized. Several recent studies support the theory that gadolinium-based contrast agents play a causative role in the development of the disease. Erythropoietin therapy and endothelial damage from surgical procedures have also been suggested as potential contributing factors.


OBJECTIVE
This study attempts to help contribute to the understanding of this novel disorder.


METHODS
We performed a retrospective chart review of 6 patients diagnosed with nephrogenic systemic fibrosis at our institution. Emphasis was placed on identification of potential putative etiologic agents including gadolinium, erythropoietin therapy, and previous surgical procedures.


RESULTS
All patients had documented exposure to gadolinium-based contrast agents. Three of the 6 patients were treated with erythropoietin, and all patients underwent a previous surgical procedure.


CONCLUSIONS
This study is limited by its small size; therefore, the findings and results may not be applicable to all patients with this disorder.


CONCLUSIONS
Our data suggest that gadolinium plays a primary role in nephrogenic systemic fibrosis and that prior surgery may be a contributory factor.
Pryor
Jennifer G
JG
Department of Pathology, University of Rochester School of Medicine and Dentistry, NY, USA.
Poggioli
George
G
Galaria
Noreen
N
Gust
Anthony
A
Robison
John
J
Samie
Faramarz
F
Hanjani
N Michelle
NM
Scott
Glynis A
GA
D000328:Adult; D000368:Aged; D003287:Contrast Media / Q000009:adverse effects; D004921:Erythropoietin / Q000009:adverse effects; D005260:Female; D005355:Fibrosis; D005682:Gadolinium / Q000009:adverse effects; D006801:Humans; D008297:Male; D008875:Middle Aged; D006435:Renal Dialysis; D051437:Renal Insufficiency / Q000150:complications* / Q000473:pathology; D012189:Retrospective Studies; D012871:Skin Diseases / Q000209:etiology* / Q000473:pathology; D013514:Surgical Procedures, Operative / Q000009:adverse effects
D003287:Contrast Media; D004921:Erythropoietin; D005682:Gadolinium