Nephrogenic Systemic Fibrosis

Abstract

Nephrogenic systemic fibrosis (NSF) is a progressive multiorgan fibrosing condition mainly caused by patients' exposure to gadolinium-based contrast agents (GBCAs) used for magnetic resonance imaging (MRI). This condition is primarily characterized by thickening of the skin and subcutaneous tissue in addition to systemic manifestations. The clinical features may involve any fibrous tissue in the body, including the internal organs such as the liver, heart, muscles, and lungs. NSF is a clinically highly variable condition. Some patients may have only mild effects on the skin, other patients may have limited movement, and rarely, the disease may be lethal.The condition was first described in 2000; however, several years later, a group of nephrologists explained the pathophysiology and the important role of GBCAs to induce this condition. It occurs in patients with acute or severe chronic renal failure with an estimated glomerular filtration rate (eGFR) of less than 30 mL/min/1.73 m^2. The condition was initially called nephrogenic fibrosing dermopathy due to the cutaneous manifestations. The term gadolinium-induced fibrosis or gadolinium-induced NSF has also been used.Patients with severe renal insufficiency are at high risk of developing this condition if GBCAs are used in MRI. The incidence rate was dramatically reduced in the last years due to strict adherence of the health care providers with the regulatory guidelines that advise the radiologists to avoid using high-risk GBCAs in severe renal failure.

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