nephrogenic systemic fibrosis; nephrogenic fibrosing dermopathy (NSF/NFD)
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Etiology
- Omniscan, a gadolinium-containing contrast agent used for magnetic resonance angiography
- erythropoietin (putative)
* gadobenate dimeglumine [Multihance] not associated with risk for nephrogenic systemic fibrosis[18]
* gadobutrol [Gadavist], gadoterate meglumine [Doterem], & gadoteridol [Prohance] may also confer low or no risk for nephrogenic systemic fibrosis[18]
Epidemiology
- occurs in patients with renal failure, > 95% on dialysis
Pathology
- involves dermis & underlying fascia
- may involve internal organs, especially skeletal muscle
- may also involve cardiac muscle, cardiomyopathy[10]
- fibrotic changes may occur in fascia, lungs, heart
- muscle biopsy may show increased numbers of myofibroblasts
- skin biopsy may show
- increased numbers of CD34 positive dendrocytes
- proliferation of fibrocytes with long dendritic processes[19]
- mucin deposition in the dermis
- increased numbers of spindle cells
- disorganized collagen bundles[10]
- increased clefts
- increased numbers of CD34 positive dendrocytes
Clinical manifestations
- onset 2 weeks-4 months after offending agent
- skin tightening, joint contractures
- symmetrical brawny, wood-like induration of upper extremities & lower extremities
- resembles eosinophilic fasciitis, but in contrast, also involves the hands & feet
- spares fingers[10]
- Raynaud's phenomonon not present
- brawny hyperpigmentation[10]
* images[14]
Laboratory
- elevated serum C-reative protein[19]
- autoantibodies NOT present
Management
- prevention:
- avoid gadolinium-based contrast agents in patients with glomerular filtration rates < 30 mL/min/1.73 m2 if possible[11]
More general terms
References
- ↑ http://www.fda.gov/medwatch/safety/2006/safety06.htm#Gadolinium
- ↑ UpToDate version 14.3 http://www.utdol.com
- ↑ Cowper SE et al, Nephrogenic fibrosing dermopathy Am J Dermatopathol 2001, 23:383 PMID: https://www.ncbi.nlm.nih.gov/pubmed/11801769
- ↑ Swartz RD et al, Nephrogenic fibrosing dermopathy; a novel cutaneous fibrosing disorder in patients with renal failure. Am J Med 2003, 114:563 PMID: https://www.ncbi.nlm.nih.gov/pubmed/12753880
- ↑ Ting WW et al, Nephrogenic fibrosing dermopathy with systemic involvement Arch Dermatol 2003, 139:903 PMID: https://www.ncbi.nlm.nih.gov/pubmed/12873886
- ↑ Jimenez SA et al, Dialysis-associated systemic fibrosis (nephrogenic fibrosing dermopathy); study of inflammatory cells and transforming growth factor beta1 expression in affected skin. Arthritis Rheum 2004, 50:2660 PMID: https://www.ncbi.nlm.nih.gov/pubmed/15334482
- ↑ Mendoza FA et al, Description of 12 cases of nephrogenic fibrosing dermopathy and review of the literature. Semin Arthritis Rheum 2006, 35:238 PMID: https://www.ncbi.nlm.nih.gov/pubmed/16461069
- ↑ Centers for Disease Control and Prevention (CDC). Nephrogenic fibrosing dermopathy associated with exposure to gadolinium-containing contrast agents--St. Louis, Missouri, 2002-2006. MMWR Morb Mortal Wkly Rep. 2007 Feb 23;56(7):137-41. PMID: https://www.ncbi.nlm.nih.gov/pubmed/17318112
- ↑ Abujudeh HH et al Nephrogenic systemic fibrosis after gadopentetate dimeglumine exposure: Case series of 36 patients. Radiology 2009 Oct; 253:81. PMID: https://www.ncbi.nlm.nih.gov/pubmed/19709997
Lee CU et al Large sample of nephrogenic systemic fibrosis cases from a single institution. Arch Dermatol 2009 Oct; 145:1095. PMID: https://www.ncbi.nlm.nih.gov/pubmed/19841395 - ↑ 10.0 10.1 10.2 10.3 10.4 Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 17. American College of Physicians, Philadelphia 2009, 2012, 2015
- ↑ 11.0 11.1 Wang Y et al. Incidence of nephrogenic systemic fibrosis after adoption of restrictive gadolinium-based contrast agent guidelines. Radiology 2011 Jul; 260:105. PMID: https://www.ncbi.nlm.nih.gov/pubmed/21586680
- ↑ Cowper SE. Nephrogenic systemic fibrosis: a review and exploration of the role of gadolinium. Adv Dermatol. 2007;23:131-54 PMID: https://www.ncbi.nlm.nih.gov/pubmed/18159899
- ↑ Chen AY, Zirwas MJ, Heffernan MP. Nephrogenic systemic fibrosis: a review. J Drugs Dermatol. 2010 Jul;9(7):829-34. PMID: https://www.ncbi.nlm.nih.gov/pubmed/20677539
- ↑ 14.0 14.1 Scheinfeld NS, Elston DM (images) Medscape: Nephrogenic Systemic Fibrosis http://emedicine.medscape.com/article/1097889-overview
- ↑ DermNet NZ. Nephrogenic fibrosing dermopathy http://www.dermnetnz.org/dermal-infiltrative/nephrogenic-fibrosing-dermopathy.html
- ↑ Thomsen HS et al Nephrogenic systemic fibrosis and gadolinium-based contrast media: updated ESUR Contrast Medium Safety Committee guidelines. Eur Radiol. 2013 Feb;23(2):307-18. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/22865271
- ↑ Manjunath V, Perazella MA. Imaging patients with kidney disease in the era of NSF: can it be done safely? Clin Nephrol. 2011 Apr;75(4):279-85. Review. PMID: https://www.ncbi.nlm.nih.gov/pubmed/21426881 Free Article
- ↑ 18.0 18.1 18.2 Brett AS Is Nephrogenic Systemic Fibrosis Following Gadolinium-Enhanced Imaging Still a Problem? NEJM Journal Watch. Jan 25, 2018 Massachusetts Medical Society (subscription needed) http://www.jwatch.org
Martin DR et al. No incidence of nephrogenic systemic fibrosis after gadobenate dimeglumine administration in patients undergoing dialysis or those with severe chronic kidney disease. Radiology 2018 Jan; 286:113 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/28731375 <Internet> http://pubs.rsna.org/doi/10.1148/radiol.2017170102 - ↑ 19.0 19.1 19.2 NEJM Knowledge+ Nephrology/Urology