Nephrogenic systemic fibrosis (NSF) after administration of gadolinium-based contrast media in AV fistula patients. Review and case report

Nephrogenic systemic fibrosis (NSF) is a rare, etiologically unclear disorder in patients with end-stage renal disease (ESRD) and is possibly caused by gadolinium-based contrast agents. NSF usually develops 2-3 months after application of such agents. Herein we present a case with an extraordinarily long time course, underscoring the requirement of certain cofactors such as chronic inflammation and accelerated atherosclerosis - a common feature in ESRD patients - for NSF development. A 52-year-old woman with ESRD for 25 years developed NSF more than 4 years after the administration of gadolinium-based contrast media. Remarkable in her past med ical history was an advanced vascular sclerosis due to a chronic inflammatory state and a secondary hyperparathyroidism. This case shows that NSF can occur any time after exposure to gadolinium-based contrast agents, which seem to be a necessary, but not the sole, cause of NSF. A review of the current literature and up-to-date recommendations are also given.