Nephrogenic systemic fibrosis in a patient on long-term hemodialysis

Abstract

Patients with renal failure sometimes develop nephrogenic systemic fibrosis (NSF) following administration of gadolinium, and a few cases have been reported in Japan. There is no definitive cure; the disease is progressive and can be fulminant. We report a case of a 54-year-old woman with multiple pathologies, including lupus nephritis at 23 years, peritoneal dialysis at 34 years, hepatocellular carcinoma at 47 years, a switch to hemodialysis, partial hepatectomy and axillo-femoral bypass grafting for severe aortic stenosis at 52 years, as well as multiple MRI exposures. One month after the last MRI including an intravenous gadolinium contrast agent (Magnevist), she developed thickening of the skin with brownish hyperpigmentation on the lower legs spreading later to all extremities, which limited joint movement and resulted in contractures. She was treated with low-dose prednisolone and cyclosporine, however, she remains at present unable to walk or extend the joints of the upper and lower extremities and needs analgesia for sharp pain in the thickened skin. Various factors including multiple exposures to gadolinium-containing MRI contrast agents, inflammatory burden, and hepatic disease might have played a role in the development of NSF. This is the case of a Japanese patient with gadopenate-dimeglumine (Magnevist) related NSF. Regardless of ethnicity and the type of contrast agent, we should be aware of the potential development of NSF in patients with renal failure.