Nephrogenic Systemic Fibrosis

Excerpt

Nephrogenic systemic fibrosis (NSF) is a progressive multiorgan fibrosing condition caused by exposure to gadolinium-based contrast agents (GBCAs) used for magnetic resonance imaging (MRI) in the setting of low glomerular filtration rate (GFR). This condition is characterized by thickening of the skin and subcutaneous tissue in addition to systemic manifestations. The clinical features may also involve skeletal muscle and any fibrous tissue in the body, including the internal organs such as the liver, heart, and lungs. NSF is a clinically highly variable condition. Some patients may have only mild effects on the skin, but many patients develop significant debility. In some patients, the disease may be lethal.

The condition was first described in 2000 when several physicians across the United States (US) noted patients with a scleroderma-type illness, and the association with GBCA administration in the setting of renal failure was solidified as more cases were presented. NSF occurs in patients with acute or severe chronic renal failure, usually stage 4 or 5 chronic kidney disease (CKD), but it has also been described in patients with stage 3 CKD. The condition was initially called nephrogenic fibrosing dermopathy due to the cutaneous manifestation. Still, the terminology changed to gadolinium-induced fibrosis or NSF as more systemic manifestations came to light. Fortunately, the incidence of this condition has significantly decreased since 2007, when the US Food and Drug Administration placed a black box warning on using gadolinium-containing products in patients with severely decreased GFR. However, 32 cases were reported annually in 2019 and 2020.