Treatment of nephrogenic systemic fibrosis: limited options but hope for the future.
Nephrogenic systemic fibrosis has now been linked to gadolinium-based contrast exposure in those with compromised kidney function. When present, symptoms can be quite devastating for the patient including severe pain and immobility. Unfortunately there is a lack of a universally effective therapy at this time and the literature, reviewed in this article, is comprised of primarily case reports and small case series allowing few conclusions to be drawn. It is widely recognized that supportive management with physical therapy and aggressive pain management is essential. Resolution of renal function in acute kidney injury appears to attenuate disease in most cases and transplantation has been associated with variable success. Therapies with anecdotal benefit include extracorporeal photopheresis and intravenous sodium thiosulfate. Other interventions have shown limited success. As the mechanism becomes more readily understood, it is hoped that targeted therapy might prove more effective than currently available remedies. In all likelihood prevention will prove to be most effective in avoiding this devastating complication.
Linfert
Douglas R
DR
Division of Nephrology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Schell
Jane O
JO
Fine
Derek M
DM
D003287:Contrast Media / Q000009:adverse effects*; D005355:Fibrosis / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy*; D005682:Gadolinium / Q000009:adverse effects*; D006801:Humans; D007167:Immunotherapy; D007676:Kidney Failure, Chronic / Q000150:complications* / Q000473:pathology / Q000628:therapy; D010789:Phototherapy; D017582:Renal Replacement Therapy
D003287:Contrast Media; D005682:Gadolinium