The outcome of patients with nephrogenic systemic fibrosis after successful kidney transplantation.

Abstract

Nephrogenic systemic fibrosis (NSF) is a debilitating disease in patients with severely diminished kidney function. Currently, no standard treatment exists but improvement has been reported after restoration of kidney function. We retrospectively studied 17 NSF patients with and without successful kidney transplantation (KTx) to evaluate the effects of KTx on NSF. Nine of the 11 KTx developed NSF pretransplant whereas two developed NSF immediately after KTx with delayed graft function. Two of the six dialysis patients had previous failed kidney transplants. Age and sex were well matched. All but one patient was dialysis dependent at the time of NSF. Median follow-up was 35 months for KTx patients and 9 months for dialysis patients. Kidney transplants achieved adequate renal function with median serum creatinine of 1.4 (0.9-2.8) mg/dL and a glomerular filtration rate of 42 (19-60) mL/min/1.73 m(2). NSF improved in 54.6% of the transplanted patients and 50% of the nontransplanted patients (p = 0.86). Two KTx patients had complete resolution of their symptoms whereas four had partial improvement. Improvement in the dialysis patients was all partial. Successful KTx did not insure improvement in NSF and in fact appeared to have no significant benefit over dialysis.

• Authors: N Leung, A Shaikh, F G Cosio, M D Griffin, S C Textor, J M Gloor, T R Schwab, T S Larson, P G Dean, M Prieto, S L Nyberg, M D Stegall, C U Lee, M R Pittelkow
• Journal: American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons (Am J Transplant) Vol. 10 Issue 3 Pg. 558-62 (Mar 2010) ISSN: 1600-6143 [Electronic] United States
• PMID: 20055804 (Publication Type: Journal Article)

Chemical References

• Creatinine

Topics

• Adult
• Aged
• Creatinine (blood)
• Disease Progression
• Female
• Follow-Up Studies
• Glomerular Filtration Rate
• Graft Survival
• Humans
• Kidney Transplantation (adverse effects, methods)
• Male
• Middle Aged
• Nephrogenic Fibrosing Dermopathy (therapy)
• Retrospective Studies
• Treatment Outcome