Response:
We thank Drs. Heinz, van der Molen, and Roditi for their valuable response. The following is the opinion of the authors and is not representative of the views or policies of our institution. The patient in this case received a gadolinium-based contrast agent (GBCA) in 2015 and was diagnosed with nephrogenic systemic fibrosis (NSF) 8 weeks later. We agree with the correspondents that linear GBCAs should not be used in patients with eGFR < 30 mL/min/1.73 m2. To date, a few cases of patients who received GBCA and developed NSF since 2009 have unfortunately continued to be reported in the literature.1-3 Our intention in publishing this case was to provide ongoing education to the medical community regarding this serious condition to ensure prevention of future cases.
We thank Dr. Böhm for her important inquiry. The patient received a histopathologic diagnosis of NSF. The report from the patient’s left dorsal forearm skin punch biopsy was read by our pathologist as “fibrosis and inflammation consistent with nephrogenic systemic fibrosis,” a diagnosis agreed upon by our colleagues in the dermatology and rheumatology departments based on the rapidity of his symptom onset and progression. While we acknowledge that this patient had other inflammatory disorders of the skin that may have coexisted with the diagnosis, after weighing the preponderance of clinical evidence in support of the biopsy results, we believe that this represents a case of NSF, which is associated with high morbidity and mortality. Thankfully, the patient in this case engaged extensively in physical and occupational therapy and is still alive nearly 4 years later. We would like to thank all the letter writers for their correspondence.
Author Affiliations: Kelley Chuang and Casey Kaneshiro are Hospitalists and Jaime Betancourt is a Pulmonologist, all in the Department of Medicine at the VA Greater Los Angeles Healthcare System in California.
Correspondence: Kelley Chuang (kelleychuang@mednet.ucla.edu)
Disclosures: The authors report no conflict of interest with regard to this article.
References
1. Aggarwal A, Froehlich AA, Essah P, Brinster N, High WA, Downs RW. Complications of nephrogenic systemic fibrosis following repeated exposure to gadolinium in a man with hypothyroidism: a case report. J Med Case Rep. 2011;5:566.
2. Fuah KW, Lim CT. Erythema nodosum masking nephrogenic systemic fibrosis as initial skin manifestation. BMC Nephrol. 2017;18(1):249.
3. Koratala A, Bhatti V. Nephrogenic systemic fibrosis. Clin Case Rep. 2017;5(7):1184-1185.