Nephrogenic systemic fibrosis - clarifying risk factors and maintaining vigilance: a literature review

Abstract

Introduction Nephrogenic systemic fibrosis (NSF) is a rare but debilitating disease, which manifests as scleroderma, leads to contractures of limbs, severe pain and may even affect vital organs. Although exact etiology is not clear, NSF is strongly associated with gadolinium-based contrast agents (GBCAs) and is mostly seen in patients with advanced kidney disease [1-3]. The first case of NSF was identified in 1997, but the relation between the disease and GBCA’s wasn’t noticed until 2006 [4,5]. Whereas NSF is potentially iatrogenic, it is important to note possible risk factors which, according to the recent literature, are less stable non-linear GBCA’s, high-dose gadolinium-enhanced MRI, dialysis, kidney transplantation in history and proinflammatory processes [6]. Aim To overview the incidence of nephrogenic systemic fibrosis in patients with renal impairment, evaluate dependence on GBCA and other related risk factor from available literature. Methods Data search was conducted using MEDLINE, ScienceDirect, Oxford journals databases. Keywords used for the research: nephrogenic systemic fibrosis, gadolinium, renal failure, MRI. Inclusion criteria: access to full articles in English, randomized controlled trials, center studies and cohorts, population with renal impairment. Exclusion criteria: conference abstracts, non-English studies.16 articles met criteria, after further inspection (only renal impaired NSF population, MRI done before NSF diagnosis) - 9 were selected for analysis and 8 were included into calculation of population. Data were expressed as percentage (%) and mean. Statistical analysis was performed using MS Office. Results In 8 included articles total population was 3 791 patients and consisted of patients with stage 3-5 of chronic kidney disease only [7–14]. 692 of them were both, diagnosed with end-stage renal disease (ESRD) and received dialysis [7,10,13]. Incidence of NSF in total pop[...].