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nephrogenic systemic fibrosis; nephrogenic fibrosing dermopathy (NSF/NFD)

Etiology: 1) Omniscan, a gadolinium-containing contrast agent used for magnetic resonance angiography 2) 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: 1) involves dermis & underlying fascia 2) may involve internal organs, especially skeletal muscle - may also involve cardiac muscle, cardiomyopathy [10] 3) fibrotic changes may occur in fascia, lungs, heart 4) muscle biopsy may show increased numbers of myofibroblasts 5) skin biopsy may show a) increased numbers of CD34 positive dendrocytes - proliferation of fibrocytes with long dendritic processes [19] b) mucin deposition in the dermis c) increased numbers of spindle cells d) disorganized collagen bundles [10] e) increased clefts Clinical manifestations: 1) onset 2 weeks-4 months after offending agent 2) skin tightening, joint contractures 3) symmetrical brawny, wood-like induration of upper extremities & lower extremities 4) resembles eosinophilic fasciitis, but in contrast, also involves the hands & feet 5) spares fingers [10] 6) Raynaud's phenomonon not present 7) 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]

General

contrast nephropathy (contrast-induced nephropathy, CIN)

References

  1. http://www.fda.gov/medwatch/safety/2006/safety06.htm#Gadolinium
  2. UpToDate version 14.3 http://www.utdol.com
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  4. Swartz RD et al, Nephrogenic fibrosing dermopathy; a novel cutaneous fibrosing disorder in patients with renal failure. Am J Med 2003, 114:563 PMID: 12753880
  5. Ting WW et al, Nephrogenic fibrosing dermopathy with systemic involvement Arch Dermatol 2003, 139:903 PMID: 12873886
  6. 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: 15334482
  7. Mendoza FA et al, Description of 12 cases of nephrogenic fibrosing dermopathy and review of the literature. Semin Arthritis Rheum 2006, 35:238 PMID: 16461069
  8. 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: 17318112
  9. Abujudeh HH et al Nephrogenic systemic fibrosis after gadopentetate dimeglumine exposure: Case series of 36 patients. Radiology 2009 Oct; 253:81. PMID: 19709997 - Lee CU et al Large sample of nephrogenic systemic fibrosis cases from a single institution. Arch Dermatol 2009 Oct; 145:1095. PMID: 19841395
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  11. Wang Y et al. Incidence of nephrogenic systemic fibrosis after adoption of restrictive gadolinium-based contrast agent guidelines. Radiology 2011 Jul; 260:105. PMID: 21586680
  12. Cowper SE. Nephrogenic systemic fibrosis: a review and exploration of the role of gadolinium. Adv Dermatol. 2007;23:131-54 PMID: 18159899
  13. Chen AY, Zirwas MJ, Heffernan MP. Nephrogenic systemic fibrosis: a review. J Drugs Dermatol. 2010 Jul;9(7):829-34. PMID: 20677539
  14. Scheinfeld NS, Elston DM (images) Medscape: Nephrogenic Systemic Fibrosis http://emedicine.medscape.com/article/1097889-overview
  15. DermNet NZ. Nephrogenic fibrosing dermopathy http://www.dermnetnz.org/dermal-infiltrative/nephrogenic-fibrosing-dermopathy.html
  16. 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: 22865271
  17. 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: 21426881 Free Article
  18. 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 PMID: 28731375 http://pubs.rsna.org/doi/10.1148/radiol.2017170102
  19. NEJM Knowledge+ Nephrology/Urology