Case Reports
Nephrogenic Systemic Fibrosis in a Patient With Multiple Inflammatory Disorders
The risk of developing nephrogenic systemic fibrosis in patients with end stage renal disease may increase with exposure to gadolinium-based...
With great interest, I read the case report by Chuang, Kaneshiro, and Betancourt.1 Patients with nephrogenic systemic fibrosis (NSF) are of special interest because the disease is still unclear as mentioned by the authors. Although new cases may occur,2 this case raises some concerns that I would like to address.
First, it would be of great interest to know the date when the patient received the high-risk gadolinium-based contrast agent (GBCA) gadoversetamide. Unfortunately, the authors did not mention the date of the injection of the GBCA that probably caused NSF. Due to the obvious association between the applications of special GBCAs in 2006, the US Food and Drug Administration (FDA) warned physicians not to inject these contrast agents in patients with compromised kidney function.3 Moreover, in 2007 the American College of Radiology (ACR) published guidelines for the safe use of GBCAs in patients with renal failure.4 Also, the European Medicines Agency (EMA) demanded that companies provide warning in product inserts about the acquisition of NSF in patients with severe kidney injury.5
Second, the clinical illustration of the case is inadequate. In the manuscript, we read that the patient acquired NSF-characteristic lesions like peau d’orange skin lesions and contractures of his extremities, but unfortunately, Chuang, Kaneshiro, and Betancourt did not provide figures that show them. On the other hand, Figure 1 shows an uncharacteristic dermal induration around inflammatory and ulcerated skin lesion (pyoderma gangrenosum).1 Such clinical signs are well known and occur perilesional of different conditions independently of NSF.6-8
Third, the histological features described as presence of fibrotic tissue in the deep dermis in Figure 2, and dermal fibrosis with thick collagen deposition in Figure 31 do not confirm the existence of NSF.
Taken together, the case presented by Chuang, Kaneshiro, and Betancourt contains some unclear aspects; therefore, it is questionable whether the published case describes a patient with NSF or not. In the current presentation, the diagnosis NSF seems to be an overestimation.
NSF still is a poorly understood disorder. Therefore, exactly documented new cases could be of clinical value when providing interesting information. Even single cases could shed some light in the darkness of the pathological mechanisms of this entity. On the other hand, we should not mix the existing cohort of published NSF cases with other scleroderma-like diseases, because this will lead to a confusion. Moreover, such a practice could inhibit the discovery of the pathophysiology of NSF.
- Ingrid Böhm, MD
Author affiliations: Ingrid Böhm is a Physician in the Department of Diagnostics, Interventional and Pediatric Radiology at the University Hospital of Bern, Inselspital, University of Bern in Bern, Switzerland.
Correspondence: Ingrid Böhm (ingrid.boehm@insel.ch)
Disclosures: The author reports no conflict of interest with regard to this article.
References
1. Chuang K, Kaneshiro C, Betancourt J. Nephrogenic systemic fibrosis in a patient with multiple inflammatory disorders. Fed Pract . 2018;35(6):40-43.
2. Larson KN, Gagnon AL, Darling MD, Patterson JW, Cropley TG. Nephrogenic systemic fibrosis manifesting a decade after exposure to gadolinium. JAMA Dermatol. 2015;151(10):1117-1120.
3. US Food and Drug Administration. A Public Health Advisory. Gadolinium-containing contrast agents for magnetic resonance imaging (MRI). http://wayback.archive-it.org/7993/20170112033022/http://www.fda.gov/Drugs/DrugSafety/PostmarketDrugSafetyInformation forPatientsandProviders/ucm053112.htm. Published June 8, 2006. Accessed March 15, 2019.
4. Kanal E, Barkovich AJ, Bell C, et al; ACR Blue Ribbon Panel on MR Safety. ACR guidance document for safe MR practices: 2007. AJR Am J Roentgenol. 2007;188(6):1447-1474.
5. European Medicines Agency. Public statement: Vasovist and nephrogenic systemic fibrosis (NSF). https://www.ema.europa.eu/en/news/public-statement-vasovist-nephrogenic-systemic-fibrosis-nsf. Published February 7, 2007. Accessed March 15, 2019.
6. Luke JC. The etiology and modern treatment of varicose ulcer. Can Med Assoc J. 1940;43(3):217-221.
7. Paulsen E, Bygum A. Keratin gel as an adjuvant in the treatment of recalcitrant pyoderma gangrenosum ulcers: a case report. Acta Derm Venereol. 2019;99(2):234-235.
8. Boehm I, Bauer R. Low-dose methotrexate controls a severe form of polyarteritis nodosa. Arch Dermatol. 2000; 136(2):167-169.
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