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Volume 63, Issue 3 , Pages 389-399, September 2010

Revisiting nephrogenic systemic fibrosis in 6 kidney transplant recipients: A single-center experience

Preliminary results of this study were presented at the Ninth Annual Congress of the French Society of Nephrology and Dialysis, Lyon, France, September 10-13, 2007.

Anne A. Lemy, MD
- Department of Nephrology, Erasme Hospital, Université Libre de Bruxelles, Brussels, Belgium
Véronique del Marmol, MD, PhD
- Department of Dermatology, Erasme Hospital, Université Libre de Bruxelles, Brussels, Belgium
Athanassios Kolivras, MD
- Department of Dermatology, Erasme Hospital, Université Libre de Bruxelles, Brussels, Belgium
Whitney A. High, MD, MEng
- Dermatology Department, University of Colorado, Aurora, Colorado
Celso Matos, MD
- Department of Radiology, Erasme Hospital, Université Libre de Bruxelles, Brussels, Belgium
Marianne Laporte, MD
- Department of Dermatology, Erasme Hospital, Université Libre de Bruxelles, Brussels, Belgium
Joëlle L. Nortier, MD, PhD
- Department of Nephrology, Erasme Hospital, Université Libre de Bruxelles, Brussels, Belgium
- Correspondence to: Joëlle Nortier, MD, PhD, Erasme Hospital, Universite Libre de Bruxelles, Route de Lennik, 808 1070 Brussels, Belgium.

Accepted 24 October 2009. published online 09 July 2010.

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 β-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.

Limitations

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 β-blockers in the course of the disease merits further investigation.

Key words: β-blockers, chronic kidney disease, collagenous colitis, erythropoiesis-stimulating agents, erythropoietin, fibrosis, gadolinium, mass spectrometry, nephrogenic fibrosing dermopathy, nephrogenic systemic fibrosis, renal transplantation, ulceration