[Gadolinium-induced systemic fibrosis in advanced kidney failure].

Area of Science:

• Nephrology
• Radiology
• Toxicology

Background:

• Nephrogenic systemic fibrosis (NSF), previously known as nephrogenic fibrosing dermopathy, is a severe systemic disease.
• NSF exclusively affects patients with end-stage renal disease or severe renal impairment.
• The exact pathogenesis of NSF remains unclear, but a strong association with gadolinium-based contrast agents (GBCAs) is observed.

Observation:

• Gadolinium-containing contrast agents are suspected to cause NSF by releasing free gadolinium into tissues.
• International drug control bodies recommend avoiding GBCAs in patients with a glomerular filtration rate (GFR) below 30 ml/min.
• This case report details the first fatal instance of NSF in Spain, involving multisystemic involvement confirmed by autopsy.

Findings:

• The patient experienced repeated exposure to gadodiamide, a GBCA, increasing the likelihood of developing NSF.
• The multisystemic involvement observed in the autopsy highlights the severe and widespread impact of the disease.
• This case underscores the critical importance of preventative measures and the limitations of current treatment options.

Implications:

• The findings emphasize the necessity of strict adherence to guidelines regarding GBCA administration in patients with renal impairment.
• This case contributes to the limited literature on NSF, particularly concerning fatal outcomes and extensive organ involvement.
• Further research into the mechanisms of gadolinium toxicity and improved diagnostic or therapeutic strategies for NSF is warranted.