A Systematic Review of 639 Patients with Biopsy-confirmed Nephrogenic Systemic Fibrosis.

Background Although nephrogenic systemic fibrosis (NSF) affects the use of gadolinium-based contrast agents (GBCAs) in MRI, there continues to be limited knowledge because of the small number of patients with NSF. Purpose To perform a systematic review of NSF. Materials and Methods PubMed database was searched by using the term "Nephrogenic systemic fibrosis" from January 2000 to February 2019. Articles reporting details on individual patients with NSF diagnosis on the basis of both clinical presentations and biopsy confirmation were included. Data were pooled and authors were contacted for clarifications. Rates of NSF were compared through 2008 versus after 2008 and for group I versus group II GBCAs, assuming equal market share. Results Included were 639 patients from 173 articles. Data regarding sex were found for 295 men and 254 women. Age at NSF symptom onset was reported for 177 patients (mean, 49 years ± 16 [standard deviation]; age range, 6-87 years). There were 529 patients with documented exposure to GBCAs including gadodiamide (n = 307), gadopentetate dimeglumine (n = 49), gadoversetamide (n = 6), gadobutrol (n = 1), gadobenate dimeglumine (n = 1), multiple (n = 41), and unknown (n = 120). Among patients with previous exposure, only seven patients were administered GBCA after 2008, yielding a lower rate of NSF after 2008 (P < .001). There were motion limitations in 70.8% (296 of 418) of patients, indicating a more serious debilitation. Associated factors reported for NSF included exposure to GBCA group I (P < .001), dialysis, proinflammatory conditions, hyperphosphatemia, ß-blockers, and epoetin. For 341 patients with follow-up, 12 patients were cured and 72 patients partially improved including one during pregnancy. Among those 84 patients reported as cured or improved, in 34 patients cure or improvement occurred after renal function restoration. Four deaths were attributed to NSF. Conclusion Although 639 patients with biopsy-confirmed nephrogenic systemic fibrosis were reported, only seven were after gadolinium-based contrast agent exposure after 2008, indicating that regulatory actions and practice changes have been effective preventive measures. Improvement and sometimes cure with renal function restoration are now possible. © RSNA, 2019 See also the editorial by Davenport in this issue.

Nephrogenic systemic fibrosis in Denmark--a nationwide investigation.

BACKGROUND: Nephrogenic systemic fibrosis is a debilitating and painful disorder with an increased stimulation of the connective tissue in the skin and systemic tissues. The disease is associated with exposure to gadolinium-based contrast agent used in magnetic resonance imaging in patients with renal impairment. METHODS: The prevalence of nephrogenic systemic fibrosis has so far never been determined at a national level. In 2009, Denmark was the first country to design a guideline for the tracing of nephrogenic systemic fibrosis patients. The aim of this paper is to communicate the main findings of this quest. RESULTS: The outcome of the nationwide investigation revealed that Denmark had 65 patients with nephrogenic systemic fibrosis and thereby the highest prevalence of nephrogenic systemic fibrosis worldwide with 65 per 5.6 million inhabitants, or 12 per million. CONCLUSIONS: The nationwide investigation in Denmark revealed the highest prevalence of NSF worldwide. This may be rooted in a high level of awareness of NSF both among doctors, politicians and, not least, the media, combined with the fact that a nationwide NSF investigation was initiated.