Nephrogenic Systemic Fibrosis

Multi system disorder seen only in patients with CKD (GFR <15; rarely with a GFR of 15–29, but FDA currently warns against using gadolinium-based contrast agents in patients with a GFR <30), acute kidney injury, and after kidney transplantation.

Nephrogenic systemic fibrosis was first recognized in hemodialysis patients during the 1990s and there is a strong link to use of contrast agents containing gadolinium. Incidence is estimated around 1–4% in the highest risk (ESRD) population that has received gadolinium, and lower in patients with less severe kidney dysfunction.

Clinical Findings

Nephrogenic systemic fibrosis affects several organ systems, including: the skin, muscles, lungs, and cardiovascular system.

The most common manifestation is a debilitating fibrosing skin disorder that can range from skin-colored to erythematous papules, which coalesce to brawny patches. The skin can be thick and woody in areas and is painful out-of-proportion to findings on examination.

Treatment

Several case reports and series have described benefit for patients after treatment with corticosteroids, photopheresis, plasmapheresis, and sodium thiosulfate. The true effectiveness of these interventions is still unclear.

REFERENCES

• Daftari Besheli L et al. Current status of nephrogenic systemic fibrosis. Clin Radiol. 2014 Jul;69(7):661–8.
• Current Medical Diagnosis & Treatment 2017. Chapter 22: Kidney Disease. Suzanne Watnick; Tonja C. Dirkx.
• Schlaudecker JD, Bernheisel CR. Gadolinium-associated nephrogenic systemic fibrosis. Am Fam Physician. 2009 Oct 1;80(7):711-4.