Ocular pathologic features and gadolinium deposition in nephrogenic systemic fibrosis.

Nephrogenic systemic fibrosis (NSF), first described in 2000 in a 15-patient case series, is an extremely debilitating, multisystemic fibrosing disorder. Since that initial report, more than 300 cases have been recognized, and their histopathologic features have been categorized. Two known associations with NSF are renal dysfunction and exposure to gadolinium (Gd)– containing magnetic resonance contrast agents (GCCAs), as first suggested by Grobner in 2006. Multiple reports have also demonstrated the deposition of insoluble Gd phosphates in tissues of patients with NSF, further confirming the importance of toxic Gd release from the chelated compounds occurring in some patients with renal failure. Ophthalmic interest in NSF stems from many reports of scleral plaques seen clinically in young patients with NSF and the appearance of “chronic scleral injection” in all cases reported by Levine et al. Our study evaluated the previously unreported ophthalmic pathologic features of 2 autopsy cases of NSF, including scanning electron microscopy with energy-dispersive x-ray spectroscopy (SEM-EDS) analysis for Gd and also for the composition of the scleral plaques.