Revisiting nephrogenic systemic fibrosis in 6 kidney transplant recipients: a single-center experience.
BACKGROUND
Nephrogenic systemic fibrosis (NSF) is a fibrotic disorder occurring in patients with renal dysfunction. Exposure to gadolinium (Gd)-based contrast agents (GBCAs) during renal impairment is associated with development of NSF.


METHODS
A cross-referenced search of kidney transplantation and radiology databases at a single institution revealed the prevalence of NSF in the transplant population. Clinical records and skin biopsy specimens from 6 patients with kidney transplant given a diagnosis of NSF were reviewed to identify contributing factors.


RESULTS
Between January 1999 and December 2006, NSF was diagnosed in 6 of 705 patients with kidney transplant (0.9%). Renal function was impaired in all patients. Of 33 patients with kidney transplant exposed to GBCAs, 5 (15.2%) developed NSF. Disease onset ranged from 7 days to 11 months after exposure to GBCAs. All 5 patients exposed to GBCAs who developed NSF were also treated with a beta-blocker and clinical improvement was observed with discontinuation. The sixth case NSF appeared unrelated to Gd, without a known exposure, and testing of tissue via mass spectrometry revealed no Gd. Symptoms of NSF in this patient disappeared after administration of darbepoetin was switched from subcutaneous to intravenous injection. One patient with NSF who manifested the highest Gd level in tissue died 22 months after disease onset.


CONCLUSIONS
The study represents the retrospective experience of only a single center.


CONCLUSIONS
NSF can develop in kidney transplant recipients with altered graft function. In these patients, exposure to GBCAs appears associated with development of NSF. The role of beta-blockers in the course of the disease merits further investigation.
Lemy
Anne A
AA
Department of Nephrology, Erasme Hospital, Université Libre de Bruxelles, Brussels, Belgium.
del Marmol
Véronique
V
Kolivras
Athanassios
A
High
Whitney A
WA
Matos
Celso
C
Laporte
Marianne
M
Nortier
Joëlle L
JL
D000328:Adult; D001707:Biopsy, Needle; D003287:Contrast Media / Q000009:adverse effects; D016208:Databases, Factual; D005260:Female; D005682:Gadolinium / Q000009:adverse effects*; D006801:Humans; D007150:Immunohistochemistry; D015994:Incidence; D016030:Kidney Transplantation / Q000706:statistics & numerical data*; D008279:Magnetic Resonance Imaging / Q000009:adverse effects / Q000379:methods; D008297:Male; D008875:Middle Aged; D054989:Nephrogenic Fibrosing Dermopathy / Q000453:epidemiology* / Q000209:etiology* / Q000473:pathology; D011379:Prognosis; D012189:Retrospective Studies; D018570:Risk Assessment
D003287:Contrast Media; D005682:Gadolinium