Copyright ©The Author(s) 2021. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Nephrol. May 25, 2021; 10(3): 29-36
Published online May 25, 2021. doi: 10.5527/wjn.v10.i3.29
Nephrogenic systemic fibrosis: A frivolous entity
Vinant Bhargava, Kulwant Singh, Priti Meena, Rupan Sanyal
Vinant Bhargava, Priti Meena, Department of Nephrology, Institute of Renal Science, SIR Ganga Ram Hospital, New Delhi 110060, India
Kulwant Singh, Department of Nephrology, Ivy Hospital, Mohali Punjab 160071, India
Rupan Sanyal, Department of Radiology, University of Alabama School of Medicine, FL, 35294-3412, United States
Author contributions: Meena P performed the majority of the writing, prepared the figures and tables; Bhargava V performed data acquisition and writing; Singh K and Sanyal R provided input in writing the paper.
Conflict-of-interest statement: There is no conflict of interest to disclose.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Corresponding author: Priti Meena, DNB, MBBS, MD, Chief Doctor, Department of Nephrology, Institute of Renal Science, SIR Ganga Ram Hospital, Sarhadi Gandhi Marg, New Delhi 110060, India.
Received: January 9, 2021
Peer-review started: January 9, 2021
First decision: March 1, 2021
Revised: March 21, 2021
Accepted: May 7, 2021
Article in press: May 7, 2021
Published online: May 25, 2021

Gadolinium-based contrast agents (GBCAs) used in magnetic resonance imaging are vital in providing enhanced quality images, essential for diagnosis and treatment. Nephrogenic systemic fibrosis (NSF) with GBCAs has been a deterrent for the physician and has led to avoidance of these agents in patients with impaired kidney function. NSF is a progressive debilitating multisystem condition described classically in patients with renal insufficiency exposed to gadolinium contrast media. It is characterized by an induration and hardening of the skin. NSF is described to first involve the extremities and can imperceptibly involve internal organs. Lack of therapeutic interventions to treat NSF makes it more challenging and warrants deep insight into the pathogenesis, risk factors and treatment strategies.

Keywords: Magnetic resonance imaging, Gadolinium-based contrast agents, Nephrogenic systemic fibrosis, Chronic kidney disease, Dialysis, End stage kidney disease

Core Tip: Nephrogenic systemic fibrosis is a chronic debilitating fibrosing disorder resulting in progressive hardening or induration of the skin. It mostly develops in patients with impaired kidney function. This review aims to provide insight into the pathogenesis, risk factors, clinical features and challenges in the management of nephrogenic systemic fibrosis.