Nephrogenic Systemic Fibrosis

Abstract

Nephrogenic systemic fibrosis is a new, rare disease of unknown cause that affects patients with renal failure. Single cases led to the suspicion of a causative role of gadodiamide that is used for magnetic resonance imaging. This study therefore reviewed all of the authors' confirmed cases of nephrogenic systemic fibrosis (n ‫؍‬ 13) with respect to clinical characteristics, gadodiamide exposure, and subsequent clinical course. It was found that all had been exposed to gadodiamide before the development of nephrogenic systemic fibrosis. The delay from exposure to first sign of the disease was 2 to 75 d (median 25 d). Odds ratio for acquiring the disease when gadodiamide exposed was 32.5 (95% confidence interval 1.9 to 549.2; P < 0.0001). Seven (54%) patients became severely disabled, and one died 21 mo after exposure. No other exposure/event than gadodiamide that was common to more than a minority of the patients could be identified. These findings indicate that gadodiamide plays a causative role in nephrogenic systemic fibrosis. , previously known as nephrogenic fibrosing dermopathy, have been reported worldwide (1,2). The appearance of this new and serious disease has triggered considerable interest as to possible causative factors, including newly introduced clinical practices. However, until now, the eliciting factor(s) has not been identified. Mendoza et al. (3) recently reviewed the clinical picture of NSF. The typical patient is middle-aged and has ESRD. Most but not all are on regular dialysis treatment. The typical course begins with subacute swelling of distal parts of the extremities and is followed in subsequent weeks by severe skin induration and sometimes anatomic extension to involve thighs, antebrachium, and lower abdomen. The skin induration may be aggressive and associated with constant pain, muscle restlessness, and loss of skin flexibility. In some cases, NSF leads to serious physical disability, including wheelchair requirement. NSF initially was observed in and thought to affect solely the skin (thus the initial term nephrogenic fibrosing dermopathy), but more recent patient reports have demonstrated that several organs may be involved. Organ involvement may explain the suspected increased mortality of patients with NSF (3). There is no established treatment for NSF, but some cases have been reported to improve after kidney transplantation, and others seem to have been treated successfully with extracorporeal photopheresis (4).At the department of nephrology, Copenhagen University Hospital at Herlev, we became aware of a formerly unknown skin disease among our patients with ESRD during 2002 through 2005. Skin biopsies supported the clinical suspicion of NSF. Some of the case stories strongly indicated that the skin changes were elicited by contrast-enhanced magnetic resonance imaging (MRI). Since late 2001, we have used this method frequently for patients with ESRD, in particular for description of iliac and lower limb arteries before kidney transplantation (5). During the past 5 to 10 yr,...

Exclusion of a Brain Lesion: Is Intravenous Contrast Administration Required after Normal Precontrast Magnetic Resonance Imaging?

“…The most frequent complaints include rashes, abnormal sensations at the injection site, nausea or vomiting, anxiety and headaches 7, 8, 9, 10, 11. However, severe late reactions, such as nephrogenic systemic fibrosis, also may be seen 35. Severe anaphylactoid reactions are estimated to occur in approximately 0.01% of human patients,7 with a mortality rate of 0.0007–0.0019% 10, 36.…”

Mechanism of NSF: New evidence challenging the prevailing theory

“…To date, it has only been reported in patients with severely impaired renal function (SIRF), endstage renal disease (ESRD), and those in acute renal failure (ARF) (2)(3)(4)(5). The many other risk factors thought to be associated with NSF include: edema (6), metabolic acidosis, thrombotic events, high-dose erythropoietin (EPO) (7,8), systemic inflammation (9), recent surgery, kidney disease (10), and gadolinium (Gd 3þ )-based contrast agent (GBCA) exposure (11,12), especially when used at relatively high dose GBCA (5,13). NSF, which was first recognized by Cowper et al (1) and linked to GBCA in 2006 (11), is characterized by induration, thickening, and tightening of the skin.…”

Visualization of external carotid artery and its branches: Non–contrast‐enhanced MR angiography using balanced steady‐state free‐precession sequence and a time‐spatial labeling inversion pulse

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